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1
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Lesort C, Breton AL, Wirbel C, Durel CA, Chastagner M, Ribereau-Gayon E, Hot A, Kanitakis J. Flare of bullous lupus erythematosus in patients treated with anifrolumab for discoid lupus erythematosus: 2 cases. JAAD Case Rep 2025; 58:99-102. [PMID: 40206105 PMCID: PMC11979904 DOI: 10.1016/j.jdcr.2024.11.045] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/11/2025] Open
Affiliation(s)
- Cécile Lesort
- Department of Dermatology, Hospices Civils de Lyon, Edouard Herriot Hospital, Lyon, France
| | - Anne-Laure Breton
- Department of Dermatology, Saint Luc Saint Joseph Hospital, Lyon, France
| | - Chloé Wirbel
- Department of Dermatology, Saint Luc Saint Joseph Hospital, Lyon, France
| | - Cécile-Audrey Durel
- Department of Internal Medicine, Saint Luc Saint Joseph Hospital, Lyon, France
| | - Marine Chastagner
- Department of Dermatology, Hospices Civils de Lyon, Edouard Herriot Hospital, Lyon, France
| | | | - Arnaud Hot
- Department of Internal Medicine, Hospices Civils de Lyon, Lyon, France
| | - Jean Kanitakis
- Department of Dermatology, Hospices Civils de Lyon, Edouard Herriot Hospital, Lyon, France
- Department of Pathology, Hospices Civils de Lyon, Lyon Sud Hospital Center, Pierre Bénite, France
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2
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Vollono L, Iorizzo M, Richert B. Current Knowledge on Nail Involvement in Autoimmune Bullous Disorders. Skin Appendage Disord 2024; 10:335-341. [PMID: 39386299 PMCID: PMC11460836 DOI: 10.1159/000538553] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2024] [Accepted: 03/13/2024] [Indexed: 10/12/2024] Open
Abstract
Background There are few studies, mainly case reports, on the involvement of the nail unit in autoimmune bullous disorders. Summary Nail involvement in autoimmune bullous disorders is a significant clinical phenomenon, marked by a range of manifestations, most often not presenting with blisters like on the skin but rather with alterations of the nail unit such as paronychia, onychomadesis, or onycholysis. This involvement is particularly notable due to the unique immunological features of the nail unit, including the expression of various antigens and the presence of Langerhans cells. Conditions like pemphigus vulgaris, bullous pemphigoid, epidermolysis bullosa acquisita, linear IgA disease, and bullous systemic lupus erythematosus can lead to nail abnormalities. The prevalence of nail manifestations varies according to the disorder, and diagnosis often relies on histopathological and immunofluorescence testing. Nail involvement correlates with disease severity and duration, sometimes serving as a herald sign. Further research is needed to guide therapeutic approaches for nail involvement in autoimmune bullous diseases. Key Messages Nail involvement in autoimmune bullous nail disorders may be confusing as there are almost never bullae. One should keep the diagnosis in mind when facing an atypical paronychia.
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Affiliation(s)
| | - Matilde Iorizzo
- Private Dermatology Practice, Bellinzona/Lugano, Switzerland
| | - Bertrand Richert
- Dermatology Departement, Saint Pierre and Brugmann University Hospitals, Université Libre de Bruxelles, Brussels, Belgium
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3
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Barker CS, Bruner E, Self S, Elston DM. U-serrated and n-serrated patterns in bullous pemphigoid, epidermolysis bullosa acquisita, and bullous lupus: A retrospective observational study. J Am Acad Dermatol 2024; 90:1282-1284. [PMID: 38368951 DOI: 10.1016/j.jaad.2024.01.076] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2023] [Revised: 11/17/2023] [Accepted: 01/10/2024] [Indexed: 02/20/2024]
Affiliation(s)
- Catherine S Barker
- Department of Internal Medicine, Medical University of South Carolina, Charleston, South Carolina.
| | - Evelyn Bruner
- Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston, South Carolina
| | - Sally Self
- Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston, South Carolina
| | - Dirk M Elston
- Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, South Carolina
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4
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Behrangi E, Jafarzadeh A, Dehghani A, Shayanfar N, Goodarzi A. Development of bullous systemic lupus erythematosus in patient treated with NB-UVB: A case report and comprehensive review of the literature. Clin Case Rep 2024; 12:e9037. [PMID: 38827943 PMCID: PMC11142903 DOI: 10.1002/ccr3.9037] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2023] [Revised: 04/26/2024] [Accepted: 04/29/2024] [Indexed: 06/05/2024] Open
Abstract
Key Clinical Message The use of phototherapy is highly effective in treating various skin diseases. In this study, the aim is to present vesicular and blister lesions in patients treated with UVB for psoriasis. It is advisable to consider the possibility of BSLE in cases of vesiculobullous lesions following phototherapy, along with other potential diagnoses. Abstract Bullous systemic lupus erythematosus (BSLE) is a rare form of cutaneous lupus erythematosus that presents as vesicles and blisters on various parts of the body. The pathological appearance of these lesions often shows subepidermal vesicles with deposits of IgG, IgM, IgA, and complement C3 in granular or linear forms under direct immunofluorescence (DIF) examination. Clinical studies demonstrate the effectiveness of phototherapy in treating various skin conditions. While several studies suggest a correlation between phototherapy and the development of vesiculobullous lesions, most of these reports are related to bullous pemphigoid, with limited research on the occurrence of BSLE following phototherapy. In this case report, vesicular and blistering lesions in a 70-year-old man undergoing UVB treatment for psoriasis are described. Pathological examination confirmed the diagnosis of bullous systemic lupus erythematosus, and the patient experienced significant improvement after treatment with dapsone tablets. A literature review was conducted on the development of vesiculobullous lesions after phototherapy, comparing different approaches presented in previous studies. Our conclusion highlights the importance of considering BSLE as a possible diagnosis in cases of vesiculobullous lesions post-phototherapy, alongside other potential conditions.
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Affiliation(s)
- Elham Behrangi
- Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of MedicineIran University of Medical Sciences (IUMS)TehranIran
- Skin and Stem Cell Research CenterTehran University of Medical SciencesTehranIran
| | - Alireza Jafarzadeh
- Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of MedicineIran University of Medical Sciences (IUMS)TehranIran
| | - Abbas Dehghani
- Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of MedicineIran University of Medical Sciences (IUMS)TehranIran
| | - Nasrin Shayanfar
- Department of Pathology, Rasool Akram Medical Complex, School of MedicineIran University of Medical SciencesTehranIran
| | - Azadeh Goodarzi
- Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of MedicineIran University of Medical Sciences (IUMS)TehranIran
- Skin and Stem Cell Research CenterTehran University of Medical SciencesTehranIran
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5
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Manjaly P, Sanchez K, Gregoire S, Ly S, Kamal K, Mostaghimi A. Superficial and Bullous Neutrophilic Dermatoses: Sneddon-Wilkinson, IgA Pemphigus, and Bullous Lupus. Dermatol Clin 2024; 42:307-315. [PMID: 38423689 DOI: 10.1016/j.det.2023.08.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/02/2024]
Abstract
Sneddon-Wilkinson disease (SWD), IgA pemphigus, and bullous systemic lupus erythematosus (BSLE) are superficial and bullous neutrophilic dermatoses. They are all characterized by sterile neutrophilic infiltrate but differ in the level of skin affected and presence of autoantibodies. Both SWD and IgA pemphigus present with grouped flaccid pustules and have epidermal involvement; it is unclear whether they are distinct or exist on a spectrum of the same disease. IgA pemphigus is distinguished from SWD by positive direct immunofluorescence showing intercellular IgA deposition. BSLE presents with tense bullae, dermal neutrophilic infiltrate, and direct immunofluorescence showing linear IgG deposition along the dermal-epidermal junction.
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Affiliation(s)
- Priya Manjaly
- Department of Dermatology, Brigham and Women's Hospital, Boston, MA 02115, USA; Boston University School of Medicine, Boston, MA 02118, USA
| | - Katherine Sanchez
- Department of Dermatology, Brigham and Women's Hospital, Boston, MA 02115, USA
| | - Samantha Gregoire
- Department of Dermatology, Brigham and Women's Hospital, Boston, MA 02115, USA
| | - Sophia Ly
- Department of Dermatology, Brigham and Women's Hospital, Boston, MA 02115, USA
| | - Kanika Kamal
- Department of Dermatology, Brigham and Women's Hospital, Boston, MA 02115, USA
| | - Arash Mostaghimi
- Department of Dermatology, Brigham and Women's Hospital, Boston, MA 02115, USA.
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6
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Yamamoto T. Role of neutrophils in cutaneous lupus erythematosus. J Dermatol 2024; 51:180-184. [PMID: 38009863 PMCID: PMC11484148 DOI: 10.1111/1346-8138.17036] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2023] [Revised: 10/24/2023] [Accepted: 10/27/2023] [Indexed: 11/29/2023]
Abstract
There are various types of cutaneous lupus erythematosus (CLE), either with or without the association of systemic lupus erythematosus (SLE). In some of the subtypes of cutaneous lupus, histopathology reveals neutrophil infiltration in the lesional skin; however, the significance of neutrophils in CLE is not yet fully elucidated. Recent studies have shown that neutrophil extracellular traps (NETs) formation by activated neutrophils is observed in several types of CLE, including lupus panniculitis, subacute lupus erythematosus, and acute lupus erythematosus, although the number of reports is small. Excessive NETosis, due to either increased NETs formation or defective clearance of NETs, may play a role in the induction of autoimmunity and autoantibody production in SLE, as well as endothelial damage, thrombus formation, and vascular damage in the lesional skin. CLE is an excessive interferon-driven autoimmune disease. Plasmacytoid dendritic cells are located in lupus erythematosus skin and contribute to the etiology of skin lesions as a main producing cell of type I interferon. Neutrophils, monocytes, and keratinocytes also produce type I interferon via several triggers. Neutrophils play an important role in the innate immune response in SLE. In this review, several types of CLE with neutrophil infiltration, as well as the role of neutrophils are discussed.
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7
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Chow KW, Sweis JJG, Alawneh D, Jetanalin P, Ascoli C, Kuschel S, Hoyer S, Braniecki M, Sweiss N. Bullous Systemic Lupus Erythematosus Successfully Treated With Intravenous Immunoglobulin and Mycophenolate Mofetil. Cureus 2023; 15:e45800. [PMID: 37876389 PMCID: PMC10590829 DOI: 10.7759/cureus.45800] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/21/2023] [Indexed: 10/26/2023] Open
Abstract
Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune blistering disorder of cutaneous lupus erythematosus (CLE) that typically manifests as an acute vesiculobullous eruption in a patient with systemic lupus erythematosus (SLE). Also, it can rarely present as the initial clinical manifestation of SLE. There is no established US Food and Drug Administration (FDA) therapy for BSLE. We report a case of a 71-year-old Hispanic woman with SLE and lupus nephritis classes III and V who presented to the hospital with a worsening rash with painful, ruptured blisters involving the upper arms, chest, and back. Our patient did not respond to topical or systemic steroids but improved rapidly to combination therapy with intravenous immunoglobulin (IVIg) and mycophenolate mofetil (MMF).
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Affiliation(s)
- Kevin W Chow
- Internal Medicine, University of Illinois at Chicago, Chicago, USA
| | | | - Diala Alawneh
- Rheumatology, University of Illinois at Chicago, Chicago, USA
| | - Pim Jetanalin
- Rheumatology, University of Illinois at Chicago, Chicago, USA
| | - Christian Ascoli
- Pulmonary and Critical Care Medicine, University of Illinois at Chicago, Chicago, USA
| | | | - Sheryl Hoyer
- Dermatology, University of Illinois at Chicago, Chicago, USA
| | | | - Nadera Sweiss
- Rheumatology, University of Illinois at Chicago, Chicago, USA
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8
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Giri G, Bagheri S. Bullous Systemic Lupus Erythematosus: A Case Report. Cureus 2023; 15:e40002. [PMID: 37416004 PMCID: PMC10322164 DOI: 10.7759/cureus.40002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/05/2023] [Indexed: 07/08/2023] Open
Abstract
Bullous systemic lupus erythematous (BSLE) is a very rare autoimmune disease characterized by vesiculobullous lesions on mostly sun-exposed areas of skin. We present a case of a 36-year-old female who developed vesiculobullous lesions after previously having poorly controlled lupus. Dapsone was added to her treatment plan, and the lesions healed in a few weeks without scarring or pigmentation.
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Affiliation(s)
- Ganesh Giri
- Medicine, Charles E Schmidt College of Medicine, Florida Atlantic University, Boca Raton, USA
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9
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Ratanapokasatit Y, Seree-Aphinan C, Chanprapaph K. Refractory Bullous Systemic Lupus Erythematosus Successfully Treated with Rituximab: A Case Report and Literature Review. Clin Cosmet Investig Dermatol 2023; 16:883-890. [PMID: 37038449 PMCID: PMC10082637 DOI: 10.2147/ccid.s403866] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/06/2023] [Accepted: 03/25/2023] [Indexed: 04/07/2023]
Abstract
Bullous systemic lupus erythematosus (BSLE) is a rare blistering skin manifestation of systemic lupus erythematosus (SLE). Dapsone is reported to be helpful in mild-to-moderate BSLE cases; however, its use may be limited or prohibited due to particular complications such as drug hypersensitivity, dose-dependent hemolytic anemia, and other significant hematologic abnormalities. Rituximab, an anti-CD20 monoclonal antibody, has been reported with off-label use in BSLE patients, but data are still limited. Hence, our objective is to explore the efficacy of rituximab among these patients. Herein, we report a 21-year-old Thai woman presented with blistering eruption on the oral cavity, scalp, trunk, and extremities for 1 month. The investigations revealed a positive direct Coomb's test, an elevated erythrocyte sedimentation rate (ESR), and a positive antinuclear antibody (ANA). Skin biopsy showed focal interface dermatitis. Direct immunofluorescence (DIF) illustrated mixed linear and granular deposition of immunoglobulin (Ig)G, IgM, IgA, and C3 along the dermo-epidermal junction (DEJ). Enzyme-linked immunosorbent assay (ELISA) showed circulating antibodies to type VII collagen. She was diagnosed with severe BSLE and autoimmune hemolytic anemia (AIHA) refractory to several oral immunosuppressants but was successfully treated with rituximab. The authors also performed a review of the literature on prior BSLE cases managed with rituximab.
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Affiliation(s)
- Yanisa Ratanapokasatit
- Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - Chutima Seree-Aphinan
- Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - Kumutnart Chanprapaph
- Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
- Correspondence: Kumutnart Chanprapaph, Division of Dermatology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Ratchathewi, Bangkok, 10400, Thailand, Tel +662-201-1141, Fax +662-201-1211, Email
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10
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Bizikova P, Olivry T, Linder K, Rybnicek J. Spontaneous autoimmune subepidermal blistering diseases in animals: a comprehensive review. BMC Vet Res 2023; 19:55. [PMID: 36849885 PMCID: PMC9969658 DOI: 10.1186/s12917-023-03597-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/28/2022] [Accepted: 01/30/2023] [Indexed: 03/01/2023] Open
Abstract
Autoimmune subepidermal blistering diseases (AISBDs) are rare skin disorders of animals that were first identified in dogs but several AISBDs are now recognised in other companion animal species. Most AISBDs in animals are homologues of the human diseases and are thought to share similar pathomechanisms of epidermal and/or mucosal blister formation caused by autoantibodies targeting structural proteins of the basement membrane zone (BMZ). Disruption of their structural function by the autoantibodies and/or recruited inflammation leads to BMZ fragility, which presents clinically as vesicles, bullae and, later, deep erosions and ulcers. Canine AISBDs are the best characterised, particularly the more common variants such as mucous membrane pemphigoid (48%), epidermolysis bullosa acquisita (EBA) (26%), and bullous pemphigoid (10%). Exceedingly rare AISBDs in the dog are junctional EBA, mixed AISBD, type-1 bullous systemic lupus erythematosus, linear IgA dermatosis, and pemphigus gestationis. The diagnosis of a specific AISBD is made by combining the clinical features (breed, age, lesion distribution) with histological evidence of subepithelial clefting, but not all AISBDs can be differentiated in this manner and specialised immunological testing is required. This latter, unfortunately, is not readily available and, therefore, the specific AISBD diagnosis often remains unconfirmed. While this limits further understanding of these diseases, it does not prevent clinicians from treating their patients, as the treatment approaches are similar for the different AISBDs in dogs. This review primarily focuses on canine AISBDs, the species for which these diseases have been best characterised, and shorter descriptions of variants in other species are also provided.
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Affiliation(s)
- Petra Bizikova
- Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, 1060 William Moore Drive, Raleigh, NC, 27607, USA.
| | - Thierry Olivry
- grid.40803.3f0000 0001 2173 6074Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, 1060 William Moore Drive, Raleigh, NC 27607 USA
| | - Keith Linder
- grid.40803.3f0000 0001 2173 6074Department of Population Health and Pathobiology, College of Veterinary Medicine, North Carolina State University, Raleigh, NC USA
| | - Jan Rybnicek
- Veterinary Dermatology and Dermatopathology Service, Padochov 175, 66491 Ivancice, Czech Republic
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11
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Kridin K, Vorobyev A, Papara C, De Luca DA, Bieber K, Ludwig RJ. Risk factors and sequelae of epidermolysis bullosa acquisita: A propensity-matched global study in 1,344 patients. Front Immunol 2023; 13:1103533. [PMID: 36776391 PMCID: PMC9910332 DOI: 10.3389/fimmu.2022.1103533] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/20/2022] [Accepted: 12/28/2022] [Indexed: 01/27/2023] Open
Abstract
Identification of risk factors and sequelae of any given disease is of key importance. For common diseases, primary prevention and disease management are based on this knowledge. For orphan diseases, identification of risk factors and sequelae has been challenging. With the advent of large databases, e.g., TriNetX, this can now be addressed. We used TriNetX to identify risk factors and sequelae of epidermolysis bullosa acquisita (EBA), a severe and orphan autoimmune disease. To date, there is only enigmatic information on EBA comorbidity. We recruited 1,344 EBA patients in the Global Collaborative Network of TriNetX. Using the "explore outcomes" function we identified 55 diagnoses with a different prevalence between EBA and no-EBA patients. We next performed propensity-matched, retrospective cohort studies in which we determined the risk of EBA development following any of the identified 55 diseases. Here, 31/55 diseases were identified as risk factors for subsequent EBA. Importantly, the highest risk for EBA were other chronic inflammatory diseases (CID), especially lupus erythematosus and lichen planus. Lastly, we determined the risk to develop any of the identified diseases after EBA diagnosis. Here, 38/55 diseases were identified as sequelae. Notably, EBA patients showed an increased risk for metabolic and cardiovascular disease, and thrombosis. Furthermore, the risk for CIDs, especially lupus erythematosus and lichen planus, was elevated. These insights into risk factors and sequelae of EBA are not only of clinical relevance, e.g., optimizing cardiovascular disease risk, but in addition, point to shared pathogenetic pathways between EBA and other inflammatory diseases.
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Affiliation(s)
- Khalaf Kridin
- Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany,Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel,Unit of Dermatology and Skin Research Laboratory, Barch Padeh Medical Center, Tiberias, Israel
| | - Artem Vorobyev
- Department of Dermatology, University Hospital Schleswig-Holstein Lübeck, Lübeck, Germany
| | - Cristian Papara
- Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany
| | - David A. De Luca
- Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany
| | - Katja Bieber
- Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany
| | - Ralf J. Ludwig
- Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany,Department of Dermatology, University Hospital Schleswig-Holstein Lübeck, Lübeck, Germany,*Correspondence: Ralf J. Ludwig,
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12
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Birke M, Glombitza M, Schneider U, Schmidt E, Klemke CD, Tratzmiller S. [Young woman with new vesiculobullous eruptions]. DERMATOLOGIE (HEIDELBERG, GERMANY) 2023; 74:386-390. [PMID: 36646854 PMCID: PMC9842400 DOI: 10.1007/s00105-022-05104-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Accepted: 12/14/2022] [Indexed: 01/18/2023]
Affiliation(s)
- Michelle Birke
- Hautklinik, Städtisches Klinikum Karlsruhe, Moltkestr. 120, 76187, Karlsruhe, Deutschland.
| | - Martin Glombitza
- Medizinische Klinik I/Innere Medizin, Städtisches Klinikum Karlsruhe, Karlsruhe, Deutschland
| | - Ulrich Schneider
- Pathologisches Institut, Städtisches Klinikum Karlsruhe, Akademisches Lehrkrankenhaus der Universität Freiburg, Karlsruhe, Deutschland
| | - Enno Schmidt
- Lübecker Institut für Experimentelle Dermatologie, Universität Lübeck, Lübeck, Deutschland
- Klinik für Dermatologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Lübeck, Deutschland
| | - Claus-Detlev Klemke
- Hautklinik, Städtisches Klinikum Karlsruhe, Moltkestr. 120, 76187, Karlsruhe, Deutschland
| | - Sabine Tratzmiller
- Hautklinik, Städtisches Klinikum Karlsruhe, Moltkestr. 120, 76187, Karlsruhe, Deutschland
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13
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Stull C, Sprow G, Werth VP. Cutaneous Involvement in Systemic Lupus Erythematosus: A Review for the Rheumatologist. J Rheumatol 2023; 50:27-35. [PMID: 36109075 PMCID: PMC10152495 DOI: 10.3899/jrheum.220089] [Citation(s) in RCA: 31] [Impact Index Per Article: 15.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/25/2022] [Indexed: 02/08/2023]
Abstract
The majority of patients with systemic lupus erythematosus (SLE) have cutaneous manifestations at some point in their disease course. The skin findings in SLE are classified as SLE-specific or SLE-nonspecific based on histopathologic findings. SLE-specific skin diseases include chronic cutaneous lupus erythematosus (CLE), subacute CLE, and acute CLE. There are subsets of skin lesions within each group and the likelihood of associated SLE varies among them. SLE-nonspecific lesions are more common in patients with SLE and tend to coincide with active systemic disease. SLE-nonspecific lesions may be seen as a feature of another disease process, including other connective tissue diseases. It is important for the rheumatologist to be familiar with the spectrum of cutaneous diseases in SLE to help prognosticate the likelihood of systemic disease and to ensure patients receive timely dermatologic care with the goal of controlling disease activity to prevent damage.
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Affiliation(s)
- Courtney Stull
- C. Stull, MD, Corporal Michael J. Crescenz VAMC, and Department of Dermatology, University of Pennsylvania, Philadelphia, and Department of Rheumatology, University of Pittsburgh Medical Center, Pittsburgh
| | - Grant Sprow
- G. Sprow, BA, V.P. Werth, MD, Corporal Michael J. Crescenz VAMC, and Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Victoria P Werth
- G. Sprow, BA, V.P. Werth, MD, Corporal Michael J. Crescenz VAMC, and Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
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14
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Nakahara Y, Yamane M, Sunada M, Aoyama Y. SARS-CoV-2 vaccine-triggered conversion from systemic lupus erythematosus (SLE) to bullous SLE and dipeptidyl peptidase 4 inhibitors-associated bullous pemphigoid. J Dermatol 2022; 50:162-165. [PMID: 36578130 PMCID: PMC9880653 DOI: 10.1111/1346-8138.16687] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/17/2022] [Accepted: 12/06/2022] [Indexed: 12/30/2022]
Abstract
Bullous systemic lupus erythematosus (BSLE) is a rare blistering disease in patients with SLE. BSLE is a heterogenous disease caused by autoantibodies to the basement membrane, mainly type VII collagen. The pathogenesis of the development of autoantibodies in BSLE remains unknown. We report a case of SLE taking dipeptidyl peptidase 4 inhibitors (DPP4i) who developed tense blister lesions after administration of SARS-CoV-2 vaccine. Initial erythematous lesion before administration of SARS-CoV-2 vaccine had not shown IgG deposition at basement membrane both direct and indirect immunofluorescence (IIF). However, the result of those examinations became positive after the administration of SARS-CoV-2 vaccine. Furthermore, IIF test results using NaCl split skin had shown positive against epidermal side. These observations suggest that SARS-CoV-2 vaccination triggered production of autoantibodies that cause bullous SLE. The present case fulfills the diagnostic criteria for both BSLE and DPP4i-associated bullous pemphigoid. Skin lesions were cleared after withdrawal of DPP4i. Therefore, physicians should ask patients who develop blisters after the vaccination whether they are taking DPP4i.
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Affiliation(s)
- Yukiko Nakahara
- Department of DermatologyKawasaki Medical SchoolOkayamaJapan
| | - Mariko Yamane
- Department of DermatologyKawasaki Medical SchoolOkayamaJapan
| | - Midori Sunada
- Department of DermatologyKawasaki Medical SchoolOkayamaJapan
| | - Yumi Aoyama
- Department of DermatologyKawasaki Medical SchoolOkayamaJapan
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15
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Téllez Arévalo AM, Quaye A, Rojas-Rodríguez LC, Poole BD, Baracaldo-Santamaría D, Tellez Freitas CM. Synthetic Pharmacotherapy for Systemic Lupus Erythematosus: Potential Mechanisms of Action, Efficacy, and Safety. MEDICINA (KAUNAS, LITHUANIA) 2022; 59:56. [PMID: 36676680 PMCID: PMC9866503 DOI: 10.3390/medicina59010056] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 10/17/2022] [Revised: 12/14/2022] [Accepted: 12/21/2022] [Indexed: 12/29/2022]
Abstract
The pharmacological treatment of systemic lupus erythematosus (SLE) aims to decrease disease activity, progression, systemic compromise, and mortality. Among the pharmacological alternatives, there are chemically synthesized drugs whose efficacy has been evaluated, but which have the potential to generate adverse events that may compromise adherence and response to treatment. Therapy selection and monitoring will depend on patient characteristics and the safety profile of each drug. The aim of this review is to provide a comprehensive understanding of the most important synthetic drugs used in the treatment of SLE, including the current treatment options (mycophenolate mofetil, azathioprine, and cyclophosphamide), review their mechanism of action, efficacy, safety, and, most importantly, provide monitoring parameters that should be considered while the patient is receiving the pharmacotherapy.
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Affiliation(s)
- Angélica María Téllez Arévalo
- Department of Physiological Sciences, School of Medicine, Pontificia Universidad Javeriana, Carrera 7 No. 40–62, Bogotá 110231, Colombia
| | - Abraham Quaye
- Department of Microbiology and Molecular Biology, Brigham Young University, Provo, UT 84602, USA
| | - Luis Carlos Rojas-Rodríguez
- Pharmacology Unit, Department of Biomedical Sciences, School of Medicine and Health Sciences, Universidad del Rosario, Bogotá 111221, Colombia
| | - Brian D. Poole
- Department of Microbiology and Molecular Biology, Brigham Young University, Provo, UT 84602, USA
| | - Daniela Baracaldo-Santamaría
- Pharmacology Unit, Department of Biomedical Sciences, School of Medicine and Health Sciences, Universidad del Rosario, Bogotá 111221, Colombia
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16
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A Rare Manifestation of Bullous Systemic Lupus Erythematosus in Children: A 10-year Retrospective Study in a Tertiary Care Hospital. Autoimmune Dis 2022; 2022:9388745. [PMID: 35911475 PMCID: PMC9325629 DOI: 10.1155/2022/9388745] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2022] [Accepted: 07/02/2022] [Indexed: 11/17/2022] Open
Abstract
Bullous systemic lupus erythematosus (BSLE) is an uncommon cutaneous presentation that occurs even less frequent in the pediatric population. A retrospective review was performed from January 2012 to December 2021 in all pediatric patients (aged <18 years) who fulfilled the diagnostic criteria for BSLE to evaluate the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence patterns, serological abnormalities, internal organ involvement, treatments, and outcomes. Among 1,415 patients with SLE, five patients were validated for the diagnosis of BSLE, accounting for 0.35%. The mean age at diagnosis was 12.2 years (standard deviation, 1.92). The clinical features of BSLE in the study population were generalized tense bullae and large extensive vesicles on the lips and perioral and mucosal areas. Pediatric BSLE in the study population revealed high SLE disease activity with multiple organ involvement. Hematologic abnormalities, serositis, and renal involvement were found in all patients, while polyarthritis (40%) and neurological abnormalities (40%) were less frequently observed. Systemic corticosteroids, intravenous immunoglobulin, immunosuppressants, antimalarials, and dapsone were prescribed in the study population. The cutaneous lesions subsided in all patients with a median clearance duration of 14 days (range, 5–56 days). BSLE in the pediatric population has auxiliary manifestations with high disease activity. Multiple organ involvement, especially hematologic abnormalities, serositis, and renal involvement, was frequently found in the study population. Although cutaneous lesions in BSLE subsided in all patients, involvement of other organs, especially renal impairment, required aggressive treatment, and long-term follow-up.
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17
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Epidermolysis bullosa acquisita. An Bras Dermatol 2022; 97:409-423. [PMID: 35701269 PMCID: PMC9263658 DOI: 10.1016/j.abd.2021.09.010] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/13/2021] [Revised: 08/19/2021] [Accepted: 09/20/2021] [Indexed: 11/24/2022] Open
Abstract
Epidermolysis bullosa acquisita is a rare autoimmune disease, characterized by the synthesis of anti-collagen VII autoantibodies, the main component of hemidesmosome anchoring fibrils. The antigen-antibody binding elicits a complex inflammatory response, which culminates in the loss of dermo-epidermal adhesion of the skin and/or mucous membranes. Skin fragility with bullae, erosions, and milia in areas of trauma characterizes the mechanobullous form of the disease. In the inflammatory form of epidermolysis bullosa acquisita, urticarial inflammatory plaques with tense bullae, similar to bullous pemphigoid, or mucosal lesions can determine permanent scars and loss of functionality in the ocular, oral, esophageal, and urogenital regions. Due to the similarity of the clinical findings of epidermolysis bullosa acquisita with other diseases of the pemphigoid group and with porphyria cutanea tarda, the diagnosis is currently confirmed mainly based on the clinical correlation with histopathological findings (pauci-inflammatory subepidermal cleavage or with a neutrophilic infiltrate) and the demonstration of the presence of anti-collagen VII IgG in situ by direct immunofluorescence, or circulating anti-collagen VII IgG through indirect immunofluorescence and/or ELISA. There is no specific therapy for epidermolysis bullosa acquisita and the response to treatment is variable, usually with complete remission in children and a worse prognosis in adults with mucosal involvement. Systemic corticosteroids and immunomodulators (colchicine and dapsone) are alternatives for the treatment of mild forms of the disease, while severe forms require the use of corticosteroid therapy associated with immunosuppressants, intravenous immunoglobulin, and rituximab.
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18
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Michalska-Jakubus M, Wdowiak-Filip A, Kowalewski C, Woźniak K, Krasowska D. Localized Blistering Eruption of the Face and Neck - A Case Study and Differential Considerations. CLINICAL, COSMETIC AND INVESTIGATIONAL DERMATOLOGY 2022; 15:271-281. [PMID: 35221704 PMCID: PMC8865756 DOI: 10.2147/ccid.s350743] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/27/2021] [Accepted: 01/25/2022] [Indexed: 11/23/2022]
Abstract
We describe a 36-year-old woman with erythematous lesions and well-tense blisters confined to the face and neck of two months history, without mucosal involvement and no triggering factors. A lesional skin biopsy showed a subepidermal blister. Direct immunofluorescence of peribullous skin identified linear deposits of IgG, IgA, and C3 complement along the basement membrane zone, whereas indirect immunofluorescence was negative. Using fluorescence overlay antigen mapping by laser scanning confocal microscopy, linear immunoglobulins deposits were found to be located above collagen IV and below laminin 332 (formerly named laminin 5), in a pattern typical of mucous membrane pemphigoid (formerly named cicatricial pemphigoid). Consequently, in terms of the clinical picture and confocal study, a rare variant of mucous membrane pemphigoid was established, namely Brunsting-Perry type. Combined therapy with oral prednisone and dapsone healed the lesions, leaving atrophic scars and milia. The paper also provides a review of previous reports on this item as well as a comprehensive differential diagnosis of facial blistering lesions.
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Affiliation(s)
| | - Anita Wdowiak-Filip
- Department of Cosmetology and Aesthetic Medicine, Medical University of Lublin, Lublin, Poland
| | - Cezary Kowalewski
- Department of Dermatology, Immunodermatology and Venereology, Medical University of Warsaw, Warsaw, Poland
| | - Katarzyna Woźniak
- Department of Dermatology, Immunodermatology and Venereology, Medical University of Warsaw, Warsaw, Poland
| | - Dorota Krasowska
- Department of Dermatology, Venereology and Pediatric Dermatology, Medical University of Lublin, Lublin, Poland
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19
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Liu Y, Liang X, Wu H, Zhuo F. Case Report: The First Reported Case of Bullous Lichen Planus-Systemic Lupus Erythematosus Overlap Syndrome. Front Med (Lausanne) 2021; 8:744592. [PMID: 34805210 PMCID: PMC8599928 DOI: 10.3389/fmed.2021.744592] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/20/2021] [Accepted: 10/04/2021] [Indexed: 11/14/2022] Open
Abstract
Introduction: Lichen planus/lupus erythematosus overlap syndrome is rarely seen in the clinic and has the characteristic clinical manifestations, histopathology, and immunopathology of lichen planus (LP) and lupus erythematosus (LE). This is the first reported case of bullous lichen planus (BLP)/systemic lupus erythematosus (SLE) overlap syndrome with hair loss as the first symptom. Case Presentation: A 48-year-old female presented with alopecia for half a year, and skin lesions accompanied by itching on her face, trunk, and limbs for 3 months. She had a history suggestive of photosensitivity. Laboratory tests and histopathology were performed for diagnosis. Histopathologic features of the upper arm and back of the hand were consistent with BLP, whereas the scalp lesion indicated LE. Laboratory examination indicated positive for antinuclear antibody (ANA) (1:160), leukopenia, increased urinary protein, decreased C3/C4, and normal BP180. The patient was given glucocorticoid combined with acitretin and immunosuppressive therapy after a definite diagnosis of BLP/SLE overlap syndrome. The lesions of the patient disappeared and some hair had regrown during the two years of follow-up. Conclusion: This is the first reported case of BLP/SLE overlap syndrome which responded well to glucocorticoids, retinoids, and immunosuppressive drugs. Multiple biopsies from characteristic lesions will guide doctors to avoid misdiagnoses and delayed treatment.
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Affiliation(s)
| | | | | | - Fenglin Zhuo
- Department of Dermatology, Beijing Friendship Hospital, Capital Medical University, Beijing, China
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20
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Mohammed Niamat Alla W, Mohamed Ali Ahmed B, Adam Essa ME, Abdalla Babker AE, Mohammed Elagib E. Vesiculobullous disease with Cutaneous Systemic Lupus Erythematosus Treated by Rituximab: A case report. Clin Case Rep 2021; 9:e04967. [PMID: 34691464 PMCID: PMC8517575 DOI: 10.1002/ccr3.4967] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/23/2021] [Revised: 10/05/2021] [Accepted: 10/06/2021] [Indexed: 11/09/2022] Open
Abstract
Vesiculobullous lesions in systemic lupus erythematosus (SLE) are a rare cutaneous manifestation of cutaneous and/or systemic LE with variable presentation. The diagnosis of SLE-associated vesiculobullous diseases remains challenging, due to the poorly defined similarities and nosology in immunohistopathological and clinical and features.
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Affiliation(s)
| | | | - Mohammed Elmujtba Adam Essa
- Department of Clinical MedicineMedical and Cancer Research InstituteKhartoumSudan
- Faculty of MedicineAlfashir UniversityAlfashirSudan
| | | | - Elnour Mohammed Elagib
- Department of RheumatologySudan Medical Specialization BoardKhartoumSudan
- Department of Internal Medicine and RheumatologyOmdurman Military hospitalKhartoumSudan
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21
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Bitar C, Menge TD, Chan MP. Cutaneous manifestations of lupus erythematosus: A practical clinicopathologic review for pathologists. Histopathology 2021; 80:233-250. [PMID: 34197657 DOI: 10.1111/his.14440] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/27/2021] [Accepted: 06/30/2021] [Indexed: 11/27/2022]
Abstract
Accurate diagnosis of connective tissue diseases is often challenging and relies on careful correlation between clinical and histopathologic features, direct immunofluorescence studies, and laboratory workup. Lupus erythematosus (LE) is a prototype of connective tissue disease with a variety of cutaneous and systemic manifestations. Microscopically, cutaneous LE is classically characterized by an interface dermatitis, although other histopathologic patterns also exist depending on the clinical presentation, location, and chronicity of the skin lesions. In this article, we review the clinical, serologic, histopathologic, and direct immunofluorescence findings in LE-specific and LE-nonspecific skin lesions, with an emphasis on lesser known variants, newly described features, and helpful ancillary studies. This review will guide general pathologists and dermatopathologists in accurately diagnosing and subclassifying cutaneous LE.
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Affiliation(s)
- Carole Bitar
- Department of Pathology, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA.,Department of Dermatology, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA
| | - Tyler D Menge
- Department of Pathology, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA.,Department of Dermatology, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA
| | - May P Chan
- Department of Pathology, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA.,Department of Dermatology, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA
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22
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Bitar C, Chan MP. Connective Tissue Diseases in the Skin: Emerging Concepts and Updates on Molecular and Immune Drivers of Disease. Surg Pathol Clin 2021; 14:237-249. [PMID: 34023103 DOI: 10.1016/j.path.2021.03.003] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
Cutaneous manifestations are common across the spectrum of autoimmune diseases. Connective tissue diseases manifesting in the skin are often difficult to classify and require integration of clinical, histopathologic, and serologic findings. This review focuses on the current understanding of the molecular and immune drivers involved in the pathogenesis of cutaneous lupus erythematosus, dermatomyositis, scleroderma/systemic sclerosis, and mixed connective tissue disease. Recent research advances have led to the emergence of new ancillary tools and useful diagnostic clues of which dermatopathologists should be aware to improve diagnostic accuracy for these diseases.
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Affiliation(s)
- Carole Bitar
- Department of Pathology, University of Michigan, 2800 Plymouth Road, NCRC Building 35, Ann Arbor, MI 48109, USA
| | - May P Chan
- Department of Pathology, University of Michigan, 2800 Plymouth Road, NCRC Building 35, Ann Arbor, MI 48109, USA.
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23
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Cooper EE, Pisano CE, Shapiro SC. Cutaneous Manifestations of "Lupus": Systemic Lupus Erythematosus and Beyond. Int J Rheumatol 2021; 2021:6610509. [PMID: 34113383 PMCID: PMC8154312 DOI: 10.1155/2021/6610509] [Citation(s) in RCA: 17] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/01/2021] [Accepted: 05/11/2021] [Indexed: 11/18/2022] Open
Abstract
Lupus, Latin for "wolf," is a term used to describe many dermatologic conditions, some of which are related to underlying systemic lupus erythematosus, while others are distinct disease processes. Cutaneous lupus erythematosus includes a wide array of visible skin manifestations and can progress to systemic lupus erythematosus in some cases. Cutaneous lupus can be subdivided into three main categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Physical exam, laboratory studies, and histopathology enable differentiation of cutaneous lupus subtypes. This differentiation is paramount as the subtype of cutaneous lupus informs upon treatment, disease monitoring, and prognostication. This review outlines the different cutaneous manifestations of lupus erythematosus and provides an update on both topical and systemic treatment options for these patients. Other conditions that utilize the term "lupus" but are not cutaneous lupus erythematosus are also discussed.
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Affiliation(s)
- Elizabeth E. Cooper
- Department of Dermatology, Dell Medical School at the University of Texas, Austin 78701, USA
| | - Catherine E. Pisano
- Department of Dermatology, Dell Medical School at the University of Texas, Austin 78701, USA
| | - Samantha C. Shapiro
- Department of Medicine, Division of Rheumatology, Dell Medical School at the University of Texas, Austin 78701, USA
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24
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Subepithelial autoimmune blistering dermatoses: Clinical features and diagnosis. J Am Acad Dermatol 2021; 85:1-14. [PMID: 33684496 DOI: 10.1016/j.jaad.2020.11.076] [Citation(s) in RCA: 23] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/04/2020] [Revised: 11/01/2020] [Accepted: 11/02/2020] [Indexed: 01/19/2023]
Abstract
Subepithelial autoimmune blistering dermatoses are a group of rare skin disorders that are characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The third article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major subepithelial autoimmune blistering dermatoses, including bullous pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, mucous membrane pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis.
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25
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Khanna U, Ellis A, Galadari A, Fernandez AP. A Refractory Blistering Eruption in a Young Woman With a Positive Antinuclear Antibody. J Clin Rheumatol 2021; 27:e62-e63. [PMID: 31609816 DOI: 10.1097/rhu.0000000000001188] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Affiliation(s)
- Urmi Khanna
- From the Department of Dermatology, Cleveland Clinic, Cleveland
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26
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Vishwajeet V, Chatterjee D, Saikia UN, Mahajan R, Radotra B, Handa S, De D. Bullous lesions in patients with cutaneous lupus erythematosus: A clinicopathologic study. J Am Acad Dermatol 2021; 85:1641-1643. [PMID: 33421476 DOI: 10.1016/j.jaad.2020.12.060] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/21/2020] [Revised: 12/19/2020] [Accepted: 12/25/2020] [Indexed: 11/19/2022]
Affiliation(s)
- Vikarn Vishwajeet
- Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Debajyoti Chatterjee
- Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
| | - Uma Nahar Saikia
- Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Rahul Mahajan
- Department of Dermatology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Bishan Radotra
- Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Sanjeev Handa
- Department of Dermatology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
| | - Dipankar De
- Department of Dermatology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
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27
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Abstract
Direct immunofluorescence (DIF) remains a valuable tool that may be underused because of perceived challenges in the interpretation, limitations, and processing of DIF specimens. The aim of this review is to provide a practical guide for appropriately incorporating DIF in a variety of clinical diseases, such as autoimmune blistering disorders. In vasculitis, the role of DIF continues to evolve, particularly in the setting of IgA vasculitis. Although typically not indicated for the workup of connective tissue disease, DIF may be helpful in cases with negative serologies, nondiagnostic histologic findings, or scarring alopecia. Practical pearls for biopsy technique, specimen handling, and storage are also discussed.
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28
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Yoshikawa N, Matsubara E, Yamamoto M, Yamazaki H, Uehara M, Kamata M, Tanaka H. Drug-induced Bullous Pemphigoid and Lupus Erythematosus Occurring under Anti-TNF-α and IL-6 Therapy in a Patient with Rheumatoid Arthritis. Intern Med 2020; 59:2611-2618. [PMID: 32641647 PMCID: PMC7662041 DOI: 10.2169/internalmedicine.4646-20] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022] Open
Abstract
A 65-year-old Japanese woman, who was diagnosed with rheumatoid arthritis and Sjögren's syndrome with various autoantibodies including anti-DNA antibody, developed bullous pemphigoid (BP) and hematological abnormalities like lupus erythematosus after adalimumab therapy. The discontinuation of adalimumab resolved those disorders but polyarthritis thereafter relapsed. The introduction of abatacept was not effective, but tocilizumab was found to be effective for polyarthritis, however, thereafter both bullous disease and severe pancytopenia developed. Discontinuation of tocilizumab was effective, but polyarthritis again developed, and baricitinib resolved it. There is an increasing number of reports of drug-induced BP and lupus erythematosus, and biologics might trigger an alteration in the pathophysiological/clinical course of rheumatic disorder.
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Affiliation(s)
- Noritada Yoshikawa
- Department of Rheumatology and Allergy, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, Japan
- Division of Rheumatology, Center for Antibody and Vaccine Therap, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, Japan
| | - Erika Matsubara
- Department of Rheumatology and Allergy, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, Japan
| | - Motohisa Yamamoto
- Department of Rheumatology and Allergy, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, Japan
- Division of Rheumatology, Center for Antibody and Vaccine Therap, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, Japan
| | - Hiroki Yamazaki
- Department of Rheumatology and Allergy, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, Japan
| | - Masaaki Uehara
- Department of Rheumatology and Allergy, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, Japan
| | - Masahiro Kamata
- Department of Dermatology, Teikyo University School of Medicine, Japan
| | - Hirotoshi Tanaka
- Department of Rheumatology and Allergy, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, Japan
- Division of Rheumatology, Center for Antibody and Vaccine Therap, IMSUT Hospital, Institute of Medical Science, The University of Tokyo, Japan
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29
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Chen CB, Kuo KL, Wang CW, Lu CW, Chung-Yee Hui R, Lu KL, Chang WC, Chen WT, Yun F, Teng YC, Lee HE, Lin JY, Ho HC, Chi MH, Yu-Wei Lin Y, Chang CJ, Lin Y, Ku CL, Hung SI, Chang YC, Chung WH. Detecting Lesional Granulysin Levels for Rapid Diagnosis of Cytotoxic T lymphocyte-Mediated Bullous Skin Disorders. THE JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE 2020; 9:1327-1337.e3. [PMID: 33039642 DOI: 10.1016/j.jaip.2020.09.048] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 03/28/2020] [Revised: 09/21/2020] [Accepted: 09/22/2020] [Indexed: 12/21/2022]
Abstract
BACKGROUND Bullous skin disorders are induced by different pathomechanisms and several are emergent, including Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). Rapid diagnostic methods for SJS/TEN or cytotoxic T-lymphocyte (CTL)-mediated bullous disorders are crucial for early treatment. Granulysin, primarily expressed by CTLs, is a specific cytotoxic protein responsible for SJS/TEN and similar skin reactions. OBJECTIVE To assess granulysin levels in blister fluids to differentiate SJS/TEN and similar CTL-mediated bullous reactions from other autoimmune bullous disorders. METHODS Using ELISA, we measured granulysin in blister fluids from patients with bullous skin disorders, including SJS/TEN, erythema multiforme major, bullous fixed-drug eruption, bullous lupus erythematosus, paraneoplastic pemphigus, pemphigus vulgaris, bullous pemphigoid, purpura fulminans-related bullae, and hand-foot syndrome/hand-foot-skin reactions. We compared serum and blister granulysin levels in patients with SJS/TEN presenting varying severity, monitoring serial granulysin levels from acute to late stages. RESULTS Overall, 144 patients presenting with bullous skin disorders were enrolled. Blister granulysin levels (mean ± SD) in CTL-mediated disorders, including TEN (n = 28; 3938.7 ± 3475.7), SJS-TEN overlapping (n = 22; 1440.4 ± 1179.6), SJS (n = 14; 542.0 ± 503.2), erythema multiforme major (n = 7; 766.3 ± 1073.7), generalized bullous fixed-drug eruption (n = 10; 720.4 ± 858.3), and localized bullous fixed-drug eruption (n = 16; 69.0 ± 56.4), were significantly higher than in non-CTL-mediated bullous disorders (P < .0001), including bullous lupus erythematosus (n = 3; 22.7 ± 20.1), paraneoplastic pemphigus (n = 3; 20.3 ± 8.6), pemphigus vulgaris (n = 3; 4.4 ± 2.8), bullous pemphigoid (n = 18; 4.0 ± 2.7), purpura fulminans (n = 4; 5.9 ± 5.5), and hand-foot syndrome/hand-foot-skin reactions (n = 6; 4.6 ± 3.5). Blister granulysin levels correlated with clinical severity of SJS/TEN (P < .0001). CONCLUSIONS Determination of blister granulysin levels is a noninvasive and useful tool for rapid differential diagnosis of SJS/TEN and other similar CTL-mediated bullous skin disorders for treatment selection.
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Affiliation(s)
- Chun-Bing Chen
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Kwei-Shan, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan; Department of Dermatology, Xiamen Chang Gung Hospital, Xiamen, China; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan; Whole-Genome Research Core Laboratory of Human Diseases, Chang Gung Memorial Hospital, Keelung, Taiwan; Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Linkou, Taiwan
| | - Kang-Ling Kuo
- Department of Dermatology, Lin Shin Hospital, Taichung, Taiwan
| | - Chuang-Wei Wang
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
| | - Chun-Wei Lu
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Kwei-Shan, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan; Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Linkou, Taiwan
| | - Rosaline Chung-Yee Hui
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan
| | - Kun-Lin Lu
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan
| | - Wan-Chun Chang
- Division of Translational Therapeutics, Department of Pediatrics, Faculty of Medicine, University of British Columbia, Vancouver, BC, Canada; British Columbia Children's Hospital Research Institute, Vancouver, BC, Canada
| | - Wei-Ti Chen
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan
| | - Fu Yun
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan
| | - Yu-Chuan Teng
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
| | - Hua-En Lee
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan
| | - Jing-Yi Lin
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan
| | - Hsin-Chun Ho
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan
| | - Min-Hui Chi
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
| | - Yang Yu-Wei Lin
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan
| | - Chee Jen Chang
- College of Medicine, Chang Gung University, Taoyuan, Taiwan; Research Services Center for Health Information, Chang Gung University, Taoyuan, Taiwan; Clinical Informatics and Medical Statistics Research Center, Chang Gung University, Taoyuan, Taiwan
| | - Yu Lin
- College of Medicine, Chang Gung University, Taoyuan, Taiwan; Research Services Center for Health Information, Chang Gung University, Taoyuan, Taiwan; Clinical Informatics and Medical Statistics Research Center, Chang Gung University, Taoyuan, Taiwan
| | - Cheng-Lung Ku
- Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Kwei-Shan, Taoyuan, Taiwan; Laboratory of Human Immunology and Infectious Disease, Graduate Institute of Clinical Medical Sciences, Chang Gung University, Taoyuan, Taiwan; Department of Nephrology, Chang Gung Memorial Hospital, Taoyuan, Taiwan
| | - Shuen-Iu Hung
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
| | - Ya-Ching Chang
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan.
| | - Wen-Hung Chung
- Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Kwei-Shan, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan; Department of Dermatology, Xiamen Chang Gung Hospital, Xiamen, China; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan; Whole-Genome Research Core Laboratory of Human Diseases, Chang Gung Memorial Hospital, Keelung, Taiwan; Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Linkou, Taiwan; Department of Dermatology, Beijing Tsinghua Chang Gung Hospital, School of Clinical Medicine, Tsinghua University, Beijing, China; Department of Dermatology, Ruijin Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China; Genomic Medicine Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taiwan.
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30
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Konstantinov NK, Pearson DR. Systemic Lupus Erythematosus: Considerations in Diagnosis and Management for the Inpatient Dermatologist. CURRENT DERMATOLOGY REPORTS 2020. [DOI: 10.1007/s13671-020-00312-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
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31
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Torres Saavedra FA, Campo LR, Mendez MV, Barreneche NM, Suaza GAV, Restrepo JDR, Martinez-Gomez M. Bullous lupus as the first manifestation of systemic lupus erythematosus in the pediatric population: A diagnostic challenge in daily practice. Lupus 2020; 29:1937-1942. [PMID: 32842868 DOI: 10.1177/0961203320950814] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
INTRODUCTION In the GLADEL cohort, the bullous lupus (BSLE) prevalence was 0.41%. However, literature on pediatric BSLE is scarce. This study described the clinical, histological, and immunological characteristics and the treatment response in a series of children with BSLE as the first clinical manifestation of pediatric SLE. METHODS The clinical, histological, and immunological characteristics of a series of 5 cases of BSLE between 2010-2019 from two reference centers in Colombia were analyzed. RESULTS All cases had bullous lesions that resolved with residual hypopigmentation. One had a focal seizure, and another arthritis with thrombocytopenia. Two had transient proteinuria with normal urinalysis. Anti-nuclear antibody titers ranged from 1:160 to 1:2560, and four were anti-dsDNA (+). Five patients had anti-RNP antibodies, and four anti-Sm antibodies. All had low C3, and 80% low C4 counts; 80% had erythrocyte sedimentation rate (ESR) ≥20 mm/hour and 60% had C-reactive protein (CRP) ≥0.5 mg/dL. All patients responded to glucocorticoids and dapsone. Histology reports and direct immunofluorescence (DIF) test showed subepidermal blisters with neutrophils in the papillary dermis and linear deposits of Igs/complement proteins in 80% of the skin biopsies. IgG/IgM was present in 5 samples. IgA was positive in 60% and C3 in 80%. CONCLUSIONS In this pediatric series, BSLE tends to have a monophasic behavior associated with neuropsychiatric, skeletal, and hematological involvement in 40% of the patients, and with good prognosis.
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Affiliation(s)
- Fabio Andrés Torres Saavedra
- Rheumatology Group GRUA, Department of Internal Medicine, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia.,Hospital Universitario San Vicente Fundación, Medellín, Colombia
| | - Lyna Ramirez Campo
- Rheumatology Group GRUA, Department of Internal Medicine, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia.,Hospital Universitario Fundación Valle del Lili, Cali, Colombia
| | - Monica Velasquez Mendez
- Rheumatology Group GRUA, Department of Internal Medicine, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia.,Hospital Universitario San Vicente Fundación, Medellín, Colombia
| | - Natalia Mejia Barreneche
- Hospital Universitario San Vicente Fundación, Medellín, Colombia.,Dermatological Research Center CIDERM, Service of Dermatology, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia
| | - Gloria Andrea Vargas Suaza
- Hospital Universitario San Vicente Fundación, Medellín, Colombia.,Dermatological Research Center CIDERM, Service of Dermatology, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia
| | - Juan David Ruiz Restrepo
- Hospital Universitario San Vicente Fundación, Medellín, Colombia.,Dermatological Research Center CIDERM, Service of Dermatology, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia
| | - Manuel Martinez-Gomez
- Hospital Universitario San Vicente Fundación, Medellín, Colombia.,Dermatological Research Center CIDERM, Service of Dermatology, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia
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32
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Bullous Lupus: An Atypical Case of Refractory Disease in a Patient with Sulfa Allergy. Case Rep Rheumatol 2020; 2020:8873337. [PMID: 32802547 PMCID: PMC7403923 DOI: 10.1155/2020/8873337] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/30/2020] [Revised: 07/14/2020] [Accepted: 07/14/2020] [Indexed: 11/30/2022] Open
Abstract
Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous autoimmune disorder characterized by rapid, widespread vesiculobullous lesions in patients with Systemic Lupus Erythematosus (SLE). BSLE can present as the initial manifestation of SLE and may be a marker of severe disease. In this case report, we present a case of a 22-year-old African American woman with BSLE and impaired renal function with subsequent nephrotic range proteinuria concerning for lupus nephritis and autoimmune hemolytic anemia, refractory to systemic corticosteroids, immunoglobulin, and mycophenolate mofetil, requiring dapsone after careful desensitization due to prior history of angioedema with sulfa drugs. This case highlights the importance of the prompt recognition of BSLE as the initial manifestations of SLE and illustrates the association of BSLE with severe disease and the benefit of concomitant use of dapsone with corticosteroids and other immunosuppressant drugs, even in patients with a history of sulfa allergy.
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33
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Fernandez-Flores A, Hermosa-Gelbard A, Pérez A, Bello JA, Cabo F. Scarring alopecia in chronic cutaneous lupus erythematosus with neutrophils: A new scenario with therapeutic connotations. J Cutan Pathol 2020; 47:976-982. [PMID: 32483922 DOI: 10.1111/cup.13764] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2020] [Revised: 05/18/2020] [Accepted: 05/25/2020] [Indexed: 11/30/2022]
Abstract
The relationship between autoinflammatory and autoimmune conditions has been demonstrated in recent decades. Several autoimmune conditions exhibit an autoinflammatory component, which can manifest in various ways. Neutrophilic dermatosis in the context of lupus erythematosus (LE) is one example. Otherwise, neutrophils are rare in LE, except for the bullous variant and nonbullous neutrophilic LE. In this paper, we describe a case of scarring alopecia due to LE that stopped responding to a treatment that had been effective for years. The biopsy specimen demonstrated the presence of neutrophils in the inflammatory infiltrate. A treatment with dapsone was prescribed and yielded rapid improvement. This first case of scarring alopecia in the context of nonbullous neutrophilic LE emphasizes the importance of the infiltrate in determining the optimal therapeutic choice.
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Affiliation(s)
- Angel Fernandez-Flores
- Department of Cellular Pathology, Hospital El Bierzo, Ponferrada, Spain.,Department of Cellular Pathology, Hospital de la Reina, Ponferrada, Spain.,Research Department, Institute for Biomedical Research of A Coruña (INIBIC), University of A Coruña (UDC) A Coruña, Spain
| | - Angela Hermosa-Gelbard
- Dermatology Department, Hospital Universitario Ramón y Cajal, Madrid, Spain.,Dermatology Department, Hospital Universitario Quirón San José, Madrid, Spain.,Dermatology Department, Hospital Universitario HM Montepríncipe, Madrid, Spain
| | - Alberto Pérez
- Department of Anatomic Pathology, Complexo Hospitalario Universitario de Ourense, Ourense, Spain
| | - José Antonio Bello
- Department of Anatomic Pathology, Complexo Hospitalario Universitario de Ourense, Ourense, Spain
| | - Fernando Cabo
- Department of Dermatology, Complexo Hospitalario Universitario de Ourense, Ourense, Spain
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34
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Fujimoto M, Asai J, Asano Y, Ishii T, Iwata Y, Kawakami T, Kodera M, Abe M, Amano M, Ikegami R, Isei T, Isogai Z, Ito T, Inoue Y, Irisawa R, Ohtsuka M, Omoto Y, Kato H, Kadono T, Kaneko S, Kanoh H, Kawaguchi M, Kukino R, Kono T, Koga M, Sakai K, Sakurai E, Sarayama Y, Shintani Y, Tanioka M, Tanizaki H, Tsujita J, Doi N, Nakanishi T, Hashimoto A, Hasegawa M, Hayashi M, Hirosaki K, Fujita H, Fujiwara H, Maekawa T, Matsuo K, Madokoro N, Motegi SI, Yatsushiro H, Yamasaki O, Yoshino Y, Pavoux AJLE, Tachibana T, Ihn H. Wound, pressure ulcer and burn guidelines - 4: Guidelines for the management of connective tissue disease/vasculitis-associated skin ulcers. J Dermatol 2020; 47:1071-1109. [PMID: 31960490 DOI: 10.1111/1346-8138.15186] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2019] [Accepted: 11/17/2019] [Indexed: 11/30/2022]
Abstract
The Japanese Dermatological Association prepared guidelines focused on the treatment of skin ulcers associated with connective tissue disease/vasculitis practical in clinical settings of dermatological care. Skin ulcers associated with connective tissue diseases or vasculitis occur on the background of a wide variety of diseases including, typically, systemic sclerosis but also systemic lupus erythematosus (SLE), dermatomyositis, rheumatoid arthritis (RA), various vasculitides and antiphospholipid antibody syndrome (APS). Therefore, in preparing the present guidelines, we considered diagnostic/therapeutic approaches appropriate for each of these disorders to be necessary and developed algorithms and clinical questions for systemic sclerosis, SLE, dermatomyositis, RA, vasculitis and APS.
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Affiliation(s)
- Manabu Fujimoto
- Department of Dermatology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan
| | - Jun Asai
- Department of Dermatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Yoshihide Asano
- Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan
| | - Takayuki Ishii
- Department of Dermatology, Toyama Prefectural Central Hospital, Toyama, Japan
| | - Yohei Iwata
- Department of Dermatology, Fujita Health University School of Medicine, Toyoake, Japan
| | - Tamihiro Kawakami
- Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Japan
| | - Masanari Kodera
- Department of Dermatology, JCHO Chukyo Hospital, Nagoya, Japan
| | | | - Masahiro Amano
- Department of Dermatology, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan
| | - Ryuta Ikegami
- Department of Dermatology, JCHO Osaka Hospital, Osaka, Japan
| | - Taiki Isei
- Department of Dermatology, National Hospital Organization Osaka National Hospital, Osaka, Japan
| | - Zenzo Isogai
- Division of Dermatology and Connective Tissue Medicine, Department of Advanced Medicine, National Center for Geriatrics and Gerontology, Obu, Japan
| | - Takaaki Ito
- Department of Dermatology, Hyogo College of Medicine, Nishinomiya, Japan
| | - Yuji Inoue
- Suizenji Dermatology Clinic, Kumamoto, Japan
| | - Ryokichi Irisawa
- Department of Dermatology, Tokyo Medical University, Tokyo, Japan
| | - Masaki Ohtsuka
- Department of Dermatology, Shizuoka Cancer Center, Shizuoka, Japan
| | - Yoichi Omoto
- Department of Dermatology, Yokkaichi Municipal Hospital, Yokkaichi, Japan
| | - Hiroshi Kato
- Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Takafumi Kadono
- Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Japan
| | - Sakae Kaneko
- Department of Dermatology, Faculty of Medicine, Shimane University, Izumo, Japan
| | - Hiroyuki Kanoh
- Department of Dermatology, Gifu University Graduate School of Medicine, Gifu, Japan
| | - Masakazu Kawaguchi
- Department of Dermatology, Yamagata University, Faculty of Medicine, Yamagata, Japan
| | | | - Takeshi Kono
- Department of Dermatology, Nippon Medical School Chiba Hokusoh Hospital, Inzei, Japan
| | - Monji Koga
- Department of Dermatology, School of Medicine, Fukuoka University, Fukuoka, Japan
| | - Keisuke Sakai
- Department of Dermatology, Minamata City General Hospital & Medical Center, Minamata, Japan
| | | | | | | | | | - Hideaki Tanizaki
- Department of Dermatology, Osaka Medical College, Takatsuki, Japan
| | - Jun Tsujita
- Department of Dermatology, Social Insurance Inatsuki Hospital, Fukuoka Prefecture Social Insurance Hospital Association, Fukuoka, Japan
| | - Naotaka Doi
- Department of Dermatology, Wakayama Medical University, Wakayama, Japan
| | - Takeshi Nakanishi
- Department of Dermatology, Shiga University of Medical Science, Otsu, Japan
| | - Akira Hashimoto
- Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - Minoru Hasegawa
- Department of Dermatology, Faculty of Medical Sciences, University of Fukui, Yoshida-gun, Japan
| | - Masahiro Hayashi
- Department of Dermatology, Yamagata University, Faculty of Medicine, Yamagata, Japan
| | | | - Hideki Fujita
- Division of Dermatological Science, Department of Dermatology, Nihon University School of Medicine, Tokyo, Japan
| | - Hiroshi Fujiwara
- Department of Dermatology, Niigata University Graduate, School of Medical and Dental Sciences, Niigata, Japan.,Department of Dermatology, Uonuma Kikan Hospital, Minamiuonuma, Japan
| | - Takeo Maekawa
- Department of Dermatology, Jichi Medical University, Shimotsuke, Japan
| | | | - Naoki Madokoro
- Department of Dermatology, MAZDA Hospital, Aki-gun, Japan
| | - Sei-Ichiro Motegi
- Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan
| | | | - Osamu Yamasaki
- Department of Dermatology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, Japan
| | - Yuichiro Yoshino
- Department of Dermatology, Japanese Red Cross Kumamoto Hospital, Kumamoto, Japan
| | | | - Takao Tachibana
- Department of Dermatology, Osaka Red Cross Hospital, Osaka, Japan
| | - Hironobu Ihn
- Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
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35
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Tintle SJ, Cruse AR, Brodell RT, Duong B. Classic Findings, Mimickers, and Distinguishing Features in Primary Blistering Skin Disease. Arch Pathol Lab Med 2019; 144:136-147. [DOI: 10.5858/arpa.2019-0175-ra] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Context.—
Blistering diseases comprise a large group of clinically polymorphic and sometimes devastating diseases. During the past few decades, we have developed an elegant understanding of the broad variety of blistering diseases and the specific histopathologic mechanism of each.
Objective.—
To review examples of the classic findings of specific blistering diseases and emphasize the importance of considering unrelated conditions that can mimic the classic finding.
Data Sources.—
This article combines data from expert review, the medical literature, and dermatology and pathology texts.
Conclusions.—
We have chosen several common examples of classic blistering diseases that are mimicked by other cutaneous conditions to highlight the basic findings in blistering conditions and the importance of clinician-to-pathologist communication.
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Affiliation(s)
- Suzanne J. Tintle
- From the Departments of Dermatology (Drs Cruse and Brodell) and Pathology (Drs Tintle, Cruse, and Brodell), and Affiliate Faculty, Dermatopathology Associates (Dr Duong), University of Mississippi Medical Center, Jackson; and the Department of Dermatology, University of Rochester Medical Center, Rochester, New York (Dr Brodell)
| | - Allison R. Cruse
- From the Departments of Dermatology (Drs Cruse and Brodell) and Pathology (Drs Tintle, Cruse, and Brodell), and Affiliate Faculty, Dermatopathology Associates (Dr Duong), University of Mississippi Medical Center, Jackson; and the Department of Dermatology, University of Rochester Medical Center, Rochester, New York (Dr Brodell)
| | - Robert T. Brodell
- From the Departments of Dermatology (Drs Cruse and Brodell) and Pathology (Drs Tintle, Cruse, and Brodell), and Affiliate Faculty, Dermatopathology Associates (Dr Duong), University of Mississippi Medical Center, Jackson; and the Department of Dermatology, University of Rochester Medical Center, Rochester, New York (Dr Brodell)
| | - Buu Duong
- From the Departments of Dermatology (Drs Cruse and Brodell) and Pathology (Drs Tintle, Cruse, and Brodell), and Affiliate Faculty, Dermatopathology Associates (Dr Duong), University of Mississippi Medical Center, Jackson; and the Department of Dermatology, University of Rochester Medical Center, Rochester, New York (Dr Brodell)
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36
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Santiago L, Mascarenhas R, Tellechea Ó, Gonçalo M. Toxic epidermal necrolysis-like subacute cutaneous lupus erythematosus associated with lung carcinoma. BMJ Case Rep 2019; 12:12/10/e231152. [DOI: 10.1136/bcr-2019-231152] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Specific vesiculobullous skin lesions in lupus erythematosus (LE) are rare and must be differentiated from toxic epidermal necrolysis (TEN), TEN-like dermatoses and other vesiculobullous conditions. We report a patient with typical subacute cutaneous lupus erythematous that progressed with large sheet-like areas of epidermal detachment and Nikolsky sign resembling TEN. She had a serological profile suggestive of underlying connective tissue disease, histological findings of interface dermatitis with a lymphocytic infiltrate, positive direct immunofluorescence, resolution with immunomodulation and lack of a culprit drug, features observed in TEN-like cutaneous lupus erythematous. Furthermore, she was diagnosed with lung carcinoma, an association that has been previously reported. Differentiating a bullous eruption in the context of pre-existing LE remains difficult requiring a thorough analysis of clinical and histopathological data.
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Rutnin S, Chanprapaph K. Vesiculobullous diseases in relation to lupus erythematosus. Clin Cosmet Investig Dermatol 2019; 12:653-667. [PMID: 31564947 PMCID: PMC6732903 DOI: 10.2147/ccid.s220906] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/26/2019] [Accepted: 08/13/2019] [Indexed: 12/13/2022]
Abstract
Vesiculobullous lesions in lupus erythematosus (LE) are a rare cutaneous manifestation of cutaneous and/or systemic LE with variable presentation. While the minor forms of LE-associated vesiculobullous disease may cause disfigurement and discomfort, the severe forms can present with hyperacute reaction and life-threatening consequences. Specific LE and aspecific cutaneous LE are defined by the presence or absence of interface change on histopathology that can be applied to vesiculobullous diseases in relation to LE. However, the diagnosis of LE-associated vesiculobullous diseases remains difficult, due to the poorly defined nosology and the similarities in clinical and immunohistopathological features among them. Herein, we thoroughly review the topic of vesiculobullous skin disorders that can be encountered in LE patients and organize them into four groups: LE-specific and aspecific vesiculobullous diseases, LE-related autoimmune bullous diseases, and LE in association to non-autoimmune conditions. We sought to provide an updated overview highlighting the pathogenesis, clinical, histological, and immunopathological features, laboratory findings, and treatments and prognosis among vesiculobullous conditions in LE.
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Affiliation(s)
- Suthinee Rutnin
- Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - Kumutnart Chanprapaph
- Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
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38
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肖 朝, 毛 华, 张 国, 郑 湘. [Bullous systemic lupus erythematosus complicated by lupus nephritis in a 12-year-old girl]. ZHONGGUO DANG DAI ER KE ZA ZHI = CHINESE JOURNAL OF CONTEMPORARY PEDIATRICS 2019; 21:836-838. [PMID: 31416512 PMCID: PMC7389897 DOI: 10.7499/j.issn.1008-8830.2019.08.018] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Subscribe] [Scholar Register] [Received: 03/04/2019] [Accepted: 05/13/2019] [Indexed: 06/10/2023]
Affiliation(s)
- 朝华 肖
- />中南大学湘雅医院儿科, 湖南 长沙 410008Department of Pediatrics, Xiangya Hospital, Central South University, Changsha 410008, China
| | - 华雄 毛
- />中南大学湘雅医院儿科, 湖南 长沙 410008Department of Pediatrics, Xiangya Hospital, Central South University, Changsha 410008, China
| | - 国元 张
- />中南大学湘雅医院儿科, 湖南 长沙 410008Department of Pediatrics, Xiangya Hospital, Central South University, Changsha 410008, China
| | - 湘榕 郑
- />中南大学湘雅医院儿科, 湖南 长沙 410008Department of Pediatrics, Xiangya Hospital, Central South University, Changsha 410008, China
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39
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Thomas RM, Colon A, Motaparthi K. Rituximab in autoimmune pemphigoid diseases: Indications, optimized regimens, and practice gaps. Clin Dermatol 2019; 38:384-396. [PMID: 32563354 DOI: 10.1016/j.clindermatol.2019.07.023] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Rituximab is a monoclonal antibody targeting CD20 on B cells with proven efficacy for pemphigus vulgaris, now an FDA-approved indication. Other autoimmune bullous diseases can be challenging to treat and have significant associated morbidity and mortality, but data supporting the use of rituximab in pemphigoid group diseases remain limited. Although rituximab demonstrates efficacy for clinical improvement and remission in pemphigoid, concern for adverse events may also limit the use of this medication. We review the current evidence fo rthe use of rituximab in pemphigoid diseases, pertinent dosing schedules and laboratory monitoring, and the associated common and rare adverse events. Review of the literature to date not only supports consideration of rituximab for treatment of refractory pemphigoid group diseases but also reflects tolerability and an acceptable safety profile.
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Affiliation(s)
- Renee M Thomas
- Department of Dermatology, University of Florida College of Medicine, Gainesville, Florida, USA
| | - Alysha Colon
- Department of Dermatology, University of Florida College of Medicine, Gainesville, Florida, USA
| | - Kiran Motaparthi
- Department of Dermatology, University of Florida College of Medicine, Gainesville, Florida, USA.
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40
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Padrão EMH, Teixeira LF, Maruta CW, Aoki V, Felipe da Silva AS, Kim EIM, Smelli LA. Bullous systemic lupus erythematosus - a case report. AUTOPSY AND CASE REPORTS 2019; 9:e2018069. [PMID: 30863736 PMCID: PMC6394362 DOI: 10.4322/acr.2018.069] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2018] [Accepted: 12/08/2018] [Indexed: 12/31/2022] Open
Abstract
Skin involvement in systemic lupus erythematosus (SLE) occurs in more than 75% of patients with this condition. Vesicles and blisters in lupus erythematosus (LE) may be present in SLE secondary to interface vacuolar changes in the epidermis, in discoid LE also secondary to vacuolar epidermal changes, and in bullous LE secondary to antibodies anti-collagen VII deposits with neutrophilic aggregates. In addition, blisters can occur due to the association of SLE with other autoimmune blistering diseases (e.g. bullous pemphigoid). BSLE is a rare blistering disease that mainly occurs in females (30–40 years old), and less frequently in children and adolescents. The most common presentation is rapid and widespread development of tense vesicles and bullae over erythematous macules or plaques. Preferential sites are: superior trunk, proximal superior limbs, and face (lips) with symmetrical distribution. Mucosal involvement is common on perioral, pharyngeal, laryngeal, and genital areas. The involvement of sun-exposed areas is not mandatory. The lesions usually progress with no scarring, but hypo or hyperchromia may be present. We report an 18-year-old female patient with blistering lesions at admission, who was diagnosed with BSLE. She was initially treated with systemic prednisone and hydroxychloroquine. Her condition evolved with relapsing lesions, which required the introduction of Dapsone. The authors emphasize the relevance of recognizing BSLE—a rare presentation of SLE—which may evolve with marked clinical presentation.
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Affiliation(s)
| | - Lucas Faria Teixeira
- Universidade de São Paulo, Medical School, Department of Internal Medicine. São Paulo, SP, Brazil
| | - Celina Wakisaka Maruta
- Universidade de São Paulo, Medical School, Department of Dermatology. São Paulo, SP, Brazil
| | - Valéria Aoki
- Universidade de São Paulo, Medical School, Department of Dermatology. São Paulo, SP, Brazil
| | - Aloisio Souza Felipe da Silva
- Universidade de São Paulo, University Hospital Pathology. São Paulo, SP, Brazil.,Universidade de São Paulo, Department of Pathology. São Paulo, SP, Brazil
| | - Elizabeth In Myung Kim
- Universidade de São Paulo, Hospital Universitário, Department of Internal Medcine. São Paulo, SP, Brazil
| | - Luciana Avena Smelli
- Universidade de São Paulo, Hospital Universitário, Department of Internal Medcine. São Paulo, SP, Brazil
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41
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Abate MS, Battle LR, Emerson AN, Gardner JM, Shalin SC. Dermatologic Urgencies and Emergencies: What Every Pathologist Should Know. Arch Pathol Lab Med 2019; 143:919-942. [PMID: 30785787 DOI: 10.5858/arpa.2018-0239-ra] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
CONTEXT.— Fatal dermatologic diseases and ones with high morbidity can occur in the inpatient setting. In such cases, prompt and accurate assessment of a bedside skin biopsy is required. This may be challenging for many pathologists who are not familiar with the complexity of skin pathology and skin terminology within the fields of dermatopathology and dermatology. OBJECTIVE.— To provide the pathologist with a practical, up-to-date, and "must-know" reference guide on dermatologic urgencies and emergencies from a real-world perspective, highlighting diagnostic pearls, diagnostic pitfalls, and commonly encountered practice gaps. This review will focus on key diseases with which every pathologist should be familiar, including angioinvasive fungal infections, Stevens-Johnson syndrome/toxic epidermal necrolysis, staph-scalded-skin syndrome, acute graft-versus-host disease, bullous pemphigoid, calciphylaxis, Sweet syndrome and its histiocytoid variant, pyoderma gangrenosum, and leukocytoclastic vasculitis, as well as those in their clinical and histopathologic differential. DATA SOURCES.— This review is based on peer-reviewed literature and our personal experiences with these diseases at major academic institutions, including one where a large number of stem cell transplants are performed. This review is unique as it represents collaborative expert opinion from both a dermatopathology and a dermatology standpoint. CONCLUSIONS.— This review outlines the critical role that the pathologist plays in the outcomes of patients with dermatologic urgencies and emergencies. Improved patient care will result from prompt and accurate histopathologic diagnoses as well as an open line of communication with the dermatologist.
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Affiliation(s)
- Mallory S Abate
- From the Department of Dermatology, Saint Louis University, St Louis, Missouri (Dr Abate); the Departments of Dermatology (Dr Battle), and Pathology (Drs Gardner and Shalin), University of Arkansas for Medical Sciences, Little Rock; and the Department of Dermatology, University of Mississippi Medical Center, Jackson (Dr Emerson)
| | - Laura R Battle
- From the Department of Dermatology, Saint Louis University, St Louis, Missouri (Dr Abate); the Departments of Dermatology (Dr Battle), and Pathology (Drs Gardner and Shalin), University of Arkansas for Medical Sciences, Little Rock; and the Department of Dermatology, University of Mississippi Medical Center, Jackson (Dr Emerson)
| | - Ashley N Emerson
- From the Department of Dermatology, Saint Louis University, St Louis, Missouri (Dr Abate); the Departments of Dermatology (Dr Battle), and Pathology (Drs Gardner and Shalin), University of Arkansas for Medical Sciences, Little Rock; and the Department of Dermatology, University of Mississippi Medical Center, Jackson (Dr Emerson)
| | - Jerad M Gardner
- From the Department of Dermatology, Saint Louis University, St Louis, Missouri (Dr Abate); the Departments of Dermatology (Dr Battle), and Pathology (Drs Gardner and Shalin), University of Arkansas for Medical Sciences, Little Rock; and the Department of Dermatology, University of Mississippi Medical Center, Jackson (Dr Emerson)
| | - Sara C Shalin
- From the Department of Dermatology, Saint Louis University, St Louis, Missouri (Dr Abate); the Departments of Dermatology (Dr Battle), and Pathology (Drs Gardner and Shalin), University of Arkansas for Medical Sciences, Little Rock; and the Department of Dermatology, University of Mississippi Medical Center, Jackson (Dr Emerson)
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Li YM, Xu H, Ma H, Chen ZQ. Crusted lesions on the face of a child. Pediatr Dermatol 2018; 35:823-824. [PMID: 30132984 PMCID: PMC6282969 DOI: 10.1111/pde.13648] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Affiliation(s)
- Yu-Mei Li
- Department of Dermatology, The Affiliated Hospital of Jiangsu University, Zhenjiang, China.,Laboratory for Regeneration Medicine, Jiangsu University, Zhenjiang, China
| | - Hui Xu
- Department of Dermatology, The Affiliated Hospital of Jiangsu University, Zhenjiang, China.,Laboratory for Regeneration Medicine, Jiangsu University, Zhenjiang, China
| | - Hong Ma
- Department of Dermatology, The Affiliated Hospital of Jiangsu University, Zhenjiang, China.,Laboratory for Regeneration Medicine, Jiangsu University, Zhenjiang, China
| | - Zhi-Qiang Chen
- Department of Dermatology, The Affiliated Hospital of Jiangsu University, Zhenjiang, China
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Bullous systemic lupus erythematosus associated with increased activity of lupus nephritis: a case report and review of the literature. Postepy Dermatol Alergol 2018; 35:431-433. [PMID: 30206461 PMCID: PMC6130133 DOI: 10.5114/ada.2018.77676] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/01/2017] [Accepted: 05/07/2017] [Indexed: 12/21/2022] Open
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Sunkara B, Roofeh D, Silver S, Pearson TL, Ettel M, McCune WJ. The devil's in the dosing: severe drug-induced liver injury in a hydroxychloroquine-naive patient with subacute cutaneous lupus erythematosus and porphyria cutanea tarda. Lupus 2018; 27:1383-1386. [PMID: 29631513 DOI: 10.1177/0961203318768884] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
A 29-year-old woman with a 1.5 year history of photosensitive skin lesions on her hands presented with a malar rash, bullous lesions on her hands, and was diagnosed with subacute lupus erythematosus after serologies revealed a positive antinuclear antibody test (1:2560), and antibodies to Ro/SSA and dsDNA. Hydroxychloroquine (400 mg/day) was prescribed and the patient developed severe drug-induced liver injury. Biopsy of her bullous skin lesions was consistent with porphyria cutanea tarda, as were her serological and urinary exams. She was successfully treated with therapeutic phlebotomy. This case identifies porphyria cutanea tarda as an important differential diagnosis for the rheumatologist to consider when evaluating patients with bullous skin lesions. Hydroxychloroquine in lower doses is an effective treatment for porphyria cutanea tarda; at doses used to treat systemic lupus erythematosus and subacute cutaneous lupus, there is a potentially life-threatening complication of hepatotoxicity.
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Affiliation(s)
- B Sunkara
- 1 Division of Rheumatology, University of Michigan Medical School, USA
| | - D Roofeh
- 1 Division of Rheumatology, University of Michigan Medical School, USA
| | - S Silver
- 2 Division of Hematology/Oncology, University of Michigan Medical School, USA
| | - T LeBleu Pearson
- 3 Department of Dermatology, University of Michigan Medical School, USA
| | - M Ettel
- 4 Department of Pathology, University of Michigan Medical School, USA
| | - W J McCune
- 1 Division of Rheumatology, University of Michigan Medical School, USA
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Hans-Bittner NR, Bittner GC, Hans G, Takita LC. Bullous systemic lupus erythematosus in a 10-year-old child. An Bras Dermatol 2018; 92:37-39. [PMID: 29267441 PMCID: PMC5726672 DOI: 10.1590/abd1806-4841.20176074] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/22/2016] [Accepted: 09/19/2016] [Indexed: 11/22/2022] Open
Abstract
Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune subepidermal
blistering disease, with few cases described in childhood. It has different
clinical-pathological features. We report a case of BSLE in a 10-year-old child
with systemic lupus erythematosus, treated with prednisone and
hydroxychloroquine. There was complete remission with dapsone, with no
recurrence of skin lesions throughout one year of follow-up. We highlight the
rarity and early age of occurrence.
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Affiliation(s)
- Nelise Ritter Hans-Bittner
- Dr. Günter Hans Department of Dermatology at Hospital Universitário Maria Aparecida Pedrossian, of the Universidade Federal de Mato Grosso do Sul (HUMAP-UFMS) - Campo Grande (MS), Brazil
| | - Guilherme Canho Bittner
- Dr. Günter Hans Department of Dermatology at Hospital Universitário Maria Aparecida Pedrossian, of the Universidade Federal de Mato Grosso do Sul (HUMAP-UFMS) - Campo Grande (MS), Brazil
| | - Günter Hans
- Dr. Günter Hans Department of Dermatology at Hospital Universitário Maria Aparecida Pedrossian, of the Universidade Federal de Mato Grosso do Sul (HUMAP-UFMS) - Campo Grande (MS), Brazil.,Discipline of Dermatology of the Faculdade de Medicina, Universidade Federal de Mato Grosso do Sul (FAMED-UFMS) - Campo Grande (MS), Brazil
| | - Luiz Carlos Takita
- Dr. Günter Hans Department of Dermatology at Hospital Universitário Maria Aparecida Pedrossian, of the Universidade Federal de Mato Grosso do Sul (HUMAP-UFMS) - Campo Grande (MS), Brazil.,Discipline of Pathology of the Faculdade de Medicina, Universidade Federal de Mato Grosso do Sul (FAMED-UFMS) - Campo Grande (MS), Brazil
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Seborrheic pemphigoid. DERMATOL SIN 2017. [DOI: 10.1016/j.dsi.2017.05.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
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47
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Li AW, Weed JG, Stamey CR, Subtil A, Tomayko MM, Antaya RJ. Bullous systemic lupus erythematosus in a 6-year-old boy. Pediatr Dermatol 2017; 34:e313-e316. [PMID: 28851077 DOI: 10.1111/pde.13258] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
Bullous systemic lupus erythematosus (BSLE) is a rare subepidermal blistering disorder characterized by an acute vesiculobullous eruption in a subset of individuals with systemic lupus erythematosus. BSLE most commonly affects young women and only rarely affects children. Herein we report a rare case of BSLE in a 6-year-old boy.
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Affiliation(s)
- Alvin W Li
- School of Medicine, Yale University, New Haven, CT, USA
| | - Jason G Weed
- School of Medicine, Yale University, New Haven, CT, USA
| | - Christopher R Stamey
- Department of Dermatology, School of Medicine, Yale University, New Haven, CT, USA
| | - Antonio Subtil
- Department of Dermatology, School of Medicine, Yale University, New Haven, CT, USA
| | - Mary M Tomayko
- Department of Dermatology, School of Medicine, Yale University, New Haven, CT, USA
| | - Richard J Antaya
- Department of Dermatology, School of Medicine, Yale University, New Haven, CT, USA.,Department of Pediatrics, School of Medicine, Yale University, New Haven, CT, USA
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Dong Y, Zhang Y, Xia L, Wang P, Chen J, Xu M, Liu X, Xia Y. The deposition of anti-DNA IgG contributes to the development of cutaneous lupus erythematosus. Immunol Lett 2017; 191:1-9. [PMID: 28899632 DOI: 10.1016/j.imlet.2017.09.003] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/08/2017] [Revised: 09/01/2017] [Accepted: 09/07/2017] [Indexed: 12/11/2022]
Abstract
Anti-DNA IgG is a hallmark of systemic lupus erythematosus and induces internal injuries in patients. It is known that cutaneous lupus erythematosus (CLE) involves the deposition of autoantibodies in the dermoepidermal junction of the skin and that anti-DNA IgG binds specifically to keratinocytes. However, the definite role of anti-DNA IgG in CLE remains unclear. The purpose of this study was to elucidate the effect of anti-DNA IgG on keratinocytes in CLE. Skin tissues were collected from patients with CLE and healthy controls. Also, murine anti-DNA IgG was incubated with frozen sections of murine skin or PAM212 keratinocytes. The chemotaxis of J774.2 macrophages was evaluated in special chambers with keratinocytes under anti-DNA IgG stimulation. Enzyme-linked immunosorbent assay, flow cytometry, Western blot, and surface plasmon resonance were used to quantitate the interaction between anti-DNA IgG and keratinocyte-related self-antigens. The results showed that anti-DNA IgG could be eluted from the lesional tissues of CLE patients, depending on the serum positivity. Murine anti-DNA IgG bound preferably to the dermoepidermal zones of normal skin and specifically to collagen III and the suppressor of cytokine signalling 1 (SOCS1) but not to Ro52. Moreover, the chemotaxis of macrophages was promoted by the incubation of anti-DNA IgG with keratinocytes. Interestingly, anti-DNA IgG exaggerated both the expression and the activation of fibroblast growth factor inducible 14 (Fn14) in keratinocytes and regulated SOCS1 signals in a time-dependent manner. In conclusion, anti-DNA IgG may contribute to the development of CLE through binding to keratinocyte-related antigens, exacerbating inflammatory infiltration, and modulating Fn14 and SOCS1 pathways.
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Affiliation(s)
- Yingying Dong
- Department of Dermatology, The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an 710004, China
| | - Yi Zhang
- Intensive Care Unit, China Gezhouba Group Central Hospital, The Third Clinical Medical College of China Three Gorges University, Yichang, 443000, China
| | - Linlin Xia
- Core Research Laboratory, The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an, 710004, China
| | - Ping Wang
- Department of Immunology & Microbiology, Wannan Medical College, Wuhu, 241001, China
| | - Jingyun Chen
- Department of Dermatology, The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an 710004, China
| | - Meifeng Xu
- Department of Dermatology, The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an 710004, China
| | - Xingyin Liu
- Department of Pathogenic Biology, Nanjing Medical University, Nanjing, 211166, China
| | - Yumin Xia
- Department of Dermatology, The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an 710004, China.
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Abstracts Presented at the 20th Joint Meeting of the International Society of Dermatopathology, March 1–2, 2017, Hilton Orlando Lake Buena Vista in the Walt Disney World Resort, Lake Buena Vista, Florida, USA. Am J Dermatopathol 2017. [DOI: 10.1097/dad.0000000000000911] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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50
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Chanprapaph K, Sawatwarakul S, Vachiramon V. A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients. Lupus 2017; 26:1278-1284. [DOI: 10.1177/0961203317699714] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/22/2023]
Abstract
Objective The aim of this study was to investigate the clinical features, laboratory findings, systemic manifestations, treatment and outcome of patients with bullous systemic lupus erythematosus in a tertiary care center in Thailand. Methods We performed a retrospective review from 2002 to 2014 of all patients who fulfilled the diagnostic criteria for bullous systemic lupus erythematosus to evaluate for the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence pattern, serological abnormalities, internal organ involvement, treatments and outcome. Results Among 5149 patients with cutaneous lupus erythematosus and/or systemic lupus erythematosus, 15 developed vesiculobullous lesions. Ten patients had validation of the diagnosis of bullous systemic lupus erythematosus, accounting for 0.19%. Bullous systemic lupus erythematosus occurred after the diagnosis of systemic lupus erythematosus in six patients with a median onset of 2.5 months (0–89). Four out of 10 patients developed bullous systemic lupus erythematosus simultaneously with systemic lupus erythematosus. Hematologic abnormalities and renal involvement were found in 100% and 90%, respectively. Polyarthritis (40%) and serositis (40%) were less frequently seen. Systemic corticosteroids, immunosuppressants, antimalarials and dapsone offered resolution of cutaneous lesions. Conclusion Bullous systemic lupus erythematosus is an uncommon presentation of systemic lupus erythematosus. Blistering can occur following or simultaneously with established systemic lupus erythematosus. We propose that clinicians should carefully search for systemic involvement, especially hematologic and renal impairment, in patients presenting with bullous systemic lupus erythematosus.
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Affiliation(s)
- K Chanprapaph
- Division of Dermatology, Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - S Sawatwarakul
- Division of Dermatology, Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - V Vachiramon
- Division of Dermatology, Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
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