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Chu KY, Yu HS, Yu S. Current and Innovated Managements for Autoimmune Bullous Skin Disorders: An Overview. J Clin Med 2022; 11:3528. [PMID: 35743598 PMCID: PMC9224787 DOI: 10.3390/jcm11123528] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/16/2022] [Revised: 06/13/2022] [Accepted: 06/17/2022] [Indexed: 11/16/2022] Open
Abstract
Autoimmune bullous skin disorders are a group of disorders characterized by the formation of numerous blisters and erosions on the skin and/or the mucosal membrane, arising from autoantibodies against the intercellular adhesion molecules and the structural proteins. They can be classified into intraepithelial or subepithelial autoimmune bullous dermatoses based on the location of the targeted antigens. These dermatoses are extremely debilitating and fatal in certain cases, depending on the degree of cutaneous and mucosal involvement. Effective treatments should be implemented promptly. Glucocorticoids serve as the first-line approach due to their rapid onset of therapeutic effects and remission of the acute phase. Nonetheless, long-term applications may lead to major adverse effects that outweigh the benefits. Hence, other adjuvant therapies are mandatory to minimize the potential harm and ameliorate the quality of life. Herein, we summarize the current therapeutic strategies and introduce promising therapies for intractable autoimmune bullous diseases.
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Affiliation(s)
- Kuan-Yu Chu
- Department of Dermatology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 807378, Taiwan;
| | - Hsin-Su Yu
- Graduate Institute of Clinical Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807378, Taiwan
| | - Sebastian Yu
- Department of Dermatology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 807378, Taiwan;
- Department of Dermatology, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807378, Taiwan
- Neuroscience Research Center, Kaohsiung Medical University, Kaohsiung 807378, Taiwan
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Dermatitis Herpetiformis: An Update on Diagnosis, Disease Monitoring, and Management. MEDICINA (KAUNAS, LITHUANIA) 2021; 57:medicina57080843. [PMID: 34441049 PMCID: PMC8400185 DOI: 10.3390/medicina57080843] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 07/19/2021] [Revised: 08/15/2021] [Accepted: 08/18/2021] [Indexed: 12/13/2022]
Abstract
Dermatitis herpetiformis (DH), Duhring disease, is caused by gluten sensitivity and affects 11.2 to 75.3 per 100,000 people in the United States and Europe with an incidence of 0.4 to 3.5 per 100,000 people per year. DH is characterized by a symmetrical blistering rash on the extensor surfaces with severe pruritus. The diagnosis continues to be made primarily by pathognomonic findings on histopathology, especially direct immunofluorescence (DIF). Recently, anti-epidermal transglutaminase (TG3) antibodies have shown to be a primary diagnostic serology, while anti-tissue transglutaminase (TG2) and other autoantibodies may be used to support the diagnosis and for disease monitoring. Newly diagnosed patients with DH should be screened and assessed for associated diseases and complications. A gluten-free diet (GFD) and dapsone are still mainstays of treatment, but other medications may be necessary for recalcitrant cases. Well-controlled DH patients, managed by a dermatologist, a gastroenterologist, and a dietician, have an excellent prognosis. Our review comprehensively details the current diagnostic methods, as well as methods used to monitor its disease course. We also describe both the traditional and novel management options reported in the literature.
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Görög A, Antiga E, Caproni M, Cianchini G, De D, Dmochowski M, Dolinsek J, Drenovska K, Feliciani C, Hervonen K, Lakos Jukic I, Kinyó Á, Koltai T, Korponay-Szabó I, Marzano AV, Patsatsi A, Rose C, Salmi T, Schmidt E, Setterfield J, Shahid M, Sitaru C, Uzun S, Valitutti F, Vassileva S, Yayli S, Sárdy M. S2k guidelines (consensus statement) for diagnosis and therapy of dermatitis herpetiformis initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol 2021; 35:1251-1277. [PMID: 34004067 DOI: 10.1111/jdv.17183] [Citation(s) in RCA: 30] [Impact Index Per Article: 7.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2020] [Accepted: 01/14/2021] [Indexed: 12/15/2022]
Abstract
INTRODUCTION Dermatitis herpetiformis (DH) is a chronic, pruritic, gluten-induced skin disorder characterized by subepidermal granular IgA deposition and a variable degree of enteropathy identical to that seen in coeliac disease. So far, there has been no European consensus about the management of DH. METHODS The guidelines were created by small subgroups of a guideline committee consisting of 26 specialists from various medical fields and one patients' representative. The members of the committee then discussed the guidelines and voted for the final version at two consensus meetings. The guidelines were developed under the support of the European Academy of Dermatology and Venereology (EADV) and in collaboration with the European Dermatology Forum (EDF). RESULTS The guidelines summarize evidence-based and expert-based recommendations (S2 level) for the management of DH (see Appendix). CONCLUSION These guidelines will improve the quality of management of DH and support dermatologists in their diagnostic and therapeutic decisions.
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Affiliation(s)
- A Görög
- Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary
| | - E Antiga
- Section of Dermatology, Department of Health Sciences, University of Florence, Florence, Italy
| | - M Caproni
- Rare Diseases Unit, Section of Dermatology, Department of Health Sciences, USL Toscana Centro, European Reference Network-Skin Member, University of Florence, Florence, Italy
| | - G Cianchini
- Department of Dermatology, Cristo Re Hospital, Rome, Italy
| | - D De
- Department of Dermatology, Postgraduate Institute of Medical Education Research, Chandigarh, India
| | - M Dmochowski
- Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznań, Poland
| | - J Dolinsek
- Gastroenterology Unit, Department of Pediatrics, University Medical Center Maribor, Maribor, Slovenia.,Medical Faculty, University of Maribor, Maribor, Slovenia
| | - K Drenovska
- Department of Dermatology and Venereology, Medical University, Sofia, Bulgaria
| | - C Feliciani
- Dermatology Unit Azienda Ospedaliero - Universitaria, Università di Parma, Parma, Italy
| | - K Hervonen
- Coeliac Disease Research Center, Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland.,Department of Dermatology, Tampere University Hospital, Tampere, Finland
| | - I Lakos Jukic
- Department of Dermatology and Venereology, University Hospital Center Zagreb, School of Medicine University of Zagreb, Zagreb, Croatia
| | - Á Kinyó
- Department of Dermatology, Venereology and Oncodermatology, University of Pécs Medical School, Pécs, Hungary
| | - T Koltai
- Association of European Coeliac Societies, Brussels, Belgium.,Hungarian Coeliac Society, Budapest, Hungary
| | - I Korponay-Szabó
- Coeliac Disease Centre, Heim Pál National Paediatric Institute, Budapest, Hungary.,Faculty of Medicine, Institute of Paediatrics, University of Debrecen, Debrecen, Hungary
| | - A V Marzano
- Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.,Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy
| | - A Patsatsi
- Autoimmune Bullous Diseases Unit, 2nd Dermatology Department, Aristotle University School of Medicine, Thessaloniki, Greece
| | - C Rose
- Dermatopathology Laboratory, Lübeck, Germany.,German Coeliac Disease Society e. V., Stuttgart, Germany
| | - T Salmi
- Coeliac Disease Research Center, Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland.,Department of Dermatology, Tampere University Hospital, Tampere, Finland
| | - E Schmidt
- Department of Dermatology, University of Lübeck, Lübeck, Germany.,Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany
| | - J Setterfield
- St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.,Centre for Host Microbiome Interactions, Faculty of Dentistry Oral & Craniofacial Sciences, King's College London, London, UK
| | - M Shahid
- Department of Dermatology and Venereology, Medical University, Sofia, Bulgaria
| | - C Sitaru
- Department of Dermatology, Faculty of Medicine, Medical Center - University of Freiburg, Freiburg, Germany.,BIOSS Centre for Biological Signalling, University of Freiburg, Freiburg, Germany
| | - S Uzun
- Department of Dermatology, Akdeniz University Faculty of Medicine, Antalya, Turkey
| | - F Valitutti
- Pediatric Unit, AOU San Giovanni di Dio e Ruggi d'Aragona, Salerno, Italy
| | - S Vassileva
- Department of Dermatology and Venereology, Medical University, Sofia, Bulgaria
| | - S Yayli
- Department of Dermatology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey
| | - M Sárdy
- Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary.,Department of Dermatology and Allergy, University Hospital of LMU, Munich, Germany
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Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN. Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy. J Am Acad Dermatol 2021; 85:18-27. [PMID: 33684494 DOI: 10.1016/j.jaad.2020.05.161] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/05/2020] [Revised: 05/17/2020] [Accepted: 05/21/2020] [Indexed: 12/18/2022]
Abstract
Subepidermal (subepithelial) autoimmune blistering dermatoses are a group of rare skin disorders characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The fourth article in this continuing medical education series presents the current validated disease activity scoring systems, serologic parameters, treatments, and clinical trials for bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, bullous systemic lupus erythematosus, anti-p200 pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis.
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Affiliation(s)
| | - Julia S Lehman
- Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
| | - Dedee F Murrell
- Department of Dermatology, St George Hospital, University of New South Wales, Sydney, Australia
| | - Michael J Camilleri
- Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
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Göbel M. 34/m mit gruppiert stehenden Vesiculae und erythematösen Papeln an den Extremitäten. Hautarzt 2019; 70:50-52. [DOI: 10.1007/s00105-018-4332-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
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6
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Graziano M, Rossi M. An update on the cutaneous manifestations of coeliac disease and non-coeliac gluten sensitivity. Int Rev Immunol 2018; 37:291-300. [PMID: 30516407 DOI: 10.1080/08830185.2018.1533008] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
INTRODUCTION Coeliac disease is a gluten-induced immune-mediated enteropathy, characterised by the expression of specific genotypes and the production of autoantibodies. The inflammatory process specifically targets the intestinal mucosa, but gastrointestinal and extraintestinal signs and symptoms can also be present. Non-coeliac gluten sensitivity (NCGS) can be diagnosed in individuals who have intestinal and/or extraintestinal symptoms related to the ingestion of gluten, but do not have autoantibodies and do not suffer from lesions in the duodenal mucosa. Among the extraintestinal manifestations, cutaneous manifestations are the most common for both diseases. PURPOSE We conducted this review to illustrate the common and uncommon features underlying the association of coeliac disease and NCGS with cutaneous manifestations related to gluten ingestion. Areas covered: The roles of innate and adaptive immunity in the cutaneous appearance of gluten sensitivity will be discussed.
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Affiliation(s)
- Mario Graziano
- a Department of Medicine, Surgery and Odontology "Scuola Medica Salernitana" , University of Salerno , Salerno , Italy
| | - Mauro Rossi
- b Institute of Food Sciences, CNR , Avellino , Italy
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7
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Abstract
Dermatitis herpetiformis (DH) is an inflammatory cutaneous disease with a chronic relapsing course, pruritic polymorphic lesions, and typical histopathological and immunopathological findings. According to several evidences, DH is considered the specific cutaneous manifestation of celiac disease, and the most recent guidelines of celiac disease have stated that, in celiac patients with a proven DH, a duodenal biopsy is unnecessary for the diagnosis. In this review, the most recent data about the diagnosis and the management of DH have been reported and discussed. In particular, in patients with clinical and/or histopathological findings suggestive for DH, the finding of granular IgA deposits along the dermal–epidermal junction or at the papillary tips by direct immunofluorescence (DIF) assay, together with positive results for anti-tissue transglutaminase antibody testing, allows the diagnosis. Thereafter, a gluten-free diet should be started in association with drugs, such as dapsone, that are able to control the skin manifestations during the first phases of the diet. In conclusion, although DH is a rare autoimmune disease with specific immunopathological alterations at the skin level, its importance goes beyond the skin itself and may have a big impact on the general health status and the quality of life of the patients.
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Affiliation(s)
- Emiliano Antiga
- Department of Surgery and Translational Medicine, Section of Dermatology, University of Florence, Florence, Italy
| | - Marzia Caproni
- Department of Surgery and Translational Medicine, Section of Dermatology, University of Florence, Florence, Italy
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8
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Di Zenzo G, Carrozzo M, Chan LS. Urban legend series: mucous membrane pemphigoid. Oral Dis 2013; 20:35-54. [DOI: 10.1111/odi.12193] [Citation(s) in RCA: 33] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2013] [Revised: 09/17/2013] [Accepted: 10/02/2013] [Indexed: 11/28/2022]
Affiliation(s)
- G Di Zenzo
- Molecular and Cell Biology Laboratory; Istituto Dermopatico dell'Immacolata; IDI-IRCCS; Rome Italy
| | - M Carrozzo
- Department of Oral Medicine; Centre for Oral Health Research; Newcastle University; Newcastle upon Tyne UK
| | - LS Chan
- Department of Dermatology and Immunology/Microbiology; University of Illinois College of Medicine; Chicago IL USA
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9
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Cutaneous manifestations of gastrointestinal disease: part II. J Am Acad Dermatol 2013; 68:211.e1-33; quiz 244-6. [PMID: 23317981 DOI: 10.1016/j.jaad.2012.10.036] [Citation(s) in RCA: 84] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2012] [Revised: 10/02/2012] [Accepted: 10/03/2012] [Indexed: 02/07/2023]
Abstract
The gastrointestinal (GI) and cutaneous organ systems are closely linked. In part I of this continuing medical education article, the intricacies of this relationship were explored as they pertained to hereditary polyposis disorders, hamartomatous disorders, and paraneoplastic disease. Part II focuses on the cutaneous system's links to inflammatory bowel disease and vascular disorders. An in-depth analysis of inflammatory bowel disease skin findings is provided to aid dermatologists in recognizing and facilitating early consultation and intervention by gastroenterologists. Cutaneous signs of inflammatory bowel disease include fissures and fistulae, erythema nodosum, pyoderma gangrenosum, pyostomatitis vegetans, oral aphthous ulcers, cutaneous polyarteritis nodosa, necrotizing vasculitis, and epidermolysis bullosa acquisita. Additional immune-mediated conditions, such as diverticulitis, bowel-associated dermatosis-arthritis syndrome, Henoch-Schönlein purpura, dermatitis herpetiformis, and Degos disease, in which the skin and GI system are mutually involved, will also be discussed. Genodermatoses common to both the GI tract and the skin include Hermansky-Pudlak syndrome, pseudoxanthoma elasticum, Ehlers-Danlos syndrome, hereditary hemorrhagic telangiectasia, and blue rubber bleb nevus syndrome. Kaposi sarcoma is a neoplastic disease with lesions involving both the skin and the gastrointestinal tract. Acrodermatitis enteropathica, a condition of zinc deficiency, likewise affects both the GI and dermatologic systems. These conditions are reviewed with updates on the genetic basis, diagnostic and screening modalities, and therapeutic options. Finally, GI complications associated with vascular disorders will also be discussed.
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Gordon RA, Mays R, Sambrano B, Mayo T, Lapolla W. Antibiotics used in nonbacterial dermatologic conditions. Dermatol Ther 2012; 25:38-54. [PMID: 22591498 DOI: 10.1111/j.1529-8019.2012.01496.x] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
The majority of nonbacterial dermatological conditions treated with antibiotics benefit from the anti-inflammatory properties of these medications, usually dapsone or tetracycline. Many other antimicrobials are used to treat noninfectious conditions. The following chapter is an overview of select noninfectious dermatological conditions for which antibiotics are used, with a focus on the most common antibiotics used for their nonantimicrobial properties.
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Abstract
Linear immunoglobulin A (IgA) bullous dermatosis, also known as linear IgA disease, is an autoimmune mucocutaneous disorder characterized by subepithelial bullae, with IgA autoantibodies directed against several different antigens in the basement membrane zone. Its immunopathologic characteristic resides in the presence of a continuous linear IgA deposit along the basement membrane zone, which is clearly visible on direct immunofluorescence. This disorder shows different clinical features and distribution when adult-onset of linear IgA disease is compared with childhood-onset. Diagnosis is achieved via clinical, histopathologic, and immunopathologic examinations. Two common therapies are dapsone and sulfapyridine, which reduce the inflammatory response and achieve disease remission in a variable period of time.
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Affiliation(s)
- Giulio Fortuna
- Department of Dermatology, Program in Epithelial Biology, Stanford University School of Medicine, CA 94305, USA
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12
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Abstract
The subepidermal immunobullous diseases are a group of autoimmune blistering disorders of the skin and mucous membranes that share the common features of autoantibody deposition and blister formation at the dermal-epidermal junction or basement membrane. This group includes bullous pemphigoid, linear IgA disease, dermatitis herpetiformis, and epidermolysis bullosa acquisita, among others. Although these disorders share some common features, each disease is unique in its clinical presentation, histopathology, and immunofluorescence patterns, which allows for accurate diagnosis and disease-specific treatment strategy. Treatment of these disorders is complex and requires expert knowledge of disease pathogenesis. We review common treatment approaches for each of these disorders.
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Affiliation(s)
- Donna A Culton
- Department of Dermatology, University of North Carolina at Chapel Hill, School of Medicine, USA.
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14
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Bolotin D, Petronic-Rosic V. Dermatitis herpetiformis. Part II. Diagnosis, management, and prognosis. J Am Acad Dermatol 2011; 64:1027-33; quiz 1033-4. [PMID: 21571168 DOI: 10.1016/j.jaad.2010.09.776] [Citation(s) in RCA: 52] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/19/2010] [Revised: 09/12/2010] [Accepted: 09/26/2010] [Indexed: 01/13/2023]
Abstract
The prompt recognition of the clinical features of dermatitis herpetiformis (DH) is important, but securing a definitive diagnosis requires further work-up. Recent advances in understanding of the immunologic basis for DH have led to the development and wider availability of serologic testing, which is rapidly becoming an essential part of the diagnosis and management of DH. Part II of this series will detail the diagnosis, treatment, and follow-up for patients with DH, and will particularly focus on recent advances in the field.
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Affiliation(s)
- Diana Bolotin
- Section of Dermatology, The University of Chicago, Chicago, Illinois 60637, USA.
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15
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Burrows N, Jones RR. Methotrexate and cyclosporin are of value in the treatment of adult linear IgA disease. J DERMATOL TREAT 2009. [DOI: 10.3109/09546639209092758] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
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Caproni M, Antiga E, Melani L, Fabbri P. Guidelines for the diagnosis and treatment of dermatitis herpetiformis. J Eur Acad Dermatol Venereol 2009; 23:633-8. [PMID: 19470076 DOI: 10.1111/j.1468-3083.2009.03188.x] [Citation(s) in RCA: 108] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/12/2023]
Abstract
Dermatitis herpetiformis is a rare disease that should be considered the cutaneous expression of a gluten-sensitive enteropathy indistinguishable from celiac disease. Dermatitis herpetiformis is often misdiagnosed and to date no guidelines for the management of dermatitis herpetiformis have been published in Literature. The present guidelines have been prepared for dermatologists by the Group for Cutaneous Immunopathology of the Italian Society of Dermatology and Venereology. They reflect the best data available at the time of preparation and the clinical experience of the authors and the members of the Italian Group for Cutaneous Immunopathology. The diagnosis of dermatitis herpetiformis is established clinically, histologically, immunopathologically and serologically. A gluten-free diet (GFD) is the treatment of choice for patients with dermatitis herpetiformis. Dapsone and/or other drugs should be used during the period until the GFD is effective. In conclusion, the present guidelines provide evidence-based guidance for the diagnosis and treatment of dermatitis herpetiformis.
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Affiliation(s)
- M Caproni
- Department of Dermatological Sciences, University of Florence, Florence, Italy
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Kenani N, Mebazaa A, Denguezli M, Ghariani N, Sriha B, Belajouza C, Nouira R. Childhood linear IgA bullous dermatosis in Tunisia. Pediatr Dermatol 2009; 26:28-33. [PMID: 19250401 DOI: 10.1111/j.1525-1470.2008.00817.x] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
The objective was to determine the demographic characteristics, the clinical features, the immuno-histological findings and response to treatment of childhood linear IgA bullous dermatosis (LABD) in Tunisia. We collected all the cases of auto-immune bullous diseases of childhood, diagnosed from January 1987 to December 2006. Based on clinical, histological, and immunofluorescent features, we identified 25 cases of LABD. Sixteen male and nine female children with a mean age of 7.5 years were identified. Clinical manifestations were characterized by a vesiculo-bullous eruption in all cases associated with mucous membrane involvement in two cases. Dapsone was the main therapy in 19 cases, associated with systemic corticosteroids in eight cases. Exclusive antibiotic therapy was successful in five cases. Sixteen of those patients had resolution of disease after a mean period of 15 months and eight patients had severe clinical presentation and required a prolonged follow-up. Childhood LABD is the most frequent bullous dermatosis in Tunisia. The majority of our patients responded rapidly to dapsone treatment and were stabilized for long time. Our cases were characterized by a minimal mucosal involvement and favorable outcome. Treatment with antibiotherapy was interesting. Erythromycin and oxacillin may be considered as an alternative therapy.
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Affiliation(s)
- Nesrine Kenani
- Department of Dermatology, Farhat Hached Hospital, Sousse University, Sousse, Tunisia
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19
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Abenavoli L, Proietti I, Leggio L, Ferrulli A, Vonghia L, Capizzi R, Rotoli M, Amerio PL, Gasbarrini G, Addolorato G. Cutaneous manifestations in celiac disease. World J Gastroenterol 2006; 12:843-52. [PMID: 16521210 PMCID: PMC4066147 DOI: 10.3748/wjg.v12.i6.843] [Citation(s) in RCA: 46] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Celiac disease (CD) is an autoimmune gluten-dependent enteropathy characterized by atrophy of intestinal villi that improves after gluten-free diet (GFD). CD is often associated with extra-intestinal manifestations; among them, several skin diseases are described in CD patients. The present review reports all CD-associated skin manifestations described in the literature and tries to analyze the possible mechanisms involved in this association. The opportunity to evaluate the possible presence of CD in patients affected by skin disorders is discussed.
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Affiliation(s)
- L Abenavoli
- Institute of Internal Medicine, Catholic University, L.go Gemelli 8, 00168 Rome, Italy
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20
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Gach JE, Wilson NJE, Wojnarowska F, Ilchyshyn A. Sulfamethoxypyridazine-responsive pemphigoid nodularis: a report of two cases. J Am Acad Dermatol 2005; 53:S101-4. [PMID: 16021154 DOI: 10.1016/j.jaad.2004.07.062] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
Pemphigoid nodularis is a chronic and uncommon variant of bullous pemphigoid. The condition is characterized by the presence of prurigo nodularis-like lesions, possible history of blistering, and immunohistochemical findings of bullous pemphigoid. These patients are often unresponsive to conventional therapy with potent topical and systemic steroids and different immunosuppressive agents used alone or in combination. We describe two cases of pemphigoid nodularis treated successfully with sulfamethoxypyridazine without any adverse effects.
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Affiliation(s)
- J E Gach
- Department of Dermatology, University Hospitals Coventry, Warwickshire NHS Trust, Coventry, United Kingdom.
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21
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Abstract
Mucous membrane pemphigoid (MMP) is a sub-epithelial vesiculobullous disorder. It is now quite evident that a number of sub-epithelial vesiculobullous disorders may produce similar clinical pictures, and also that a range of variants of MMP exist, with antibodies directed against various hemidesmosomal components or components of the epithelial basement membrane. The term immune-mediated sub-epithelial blistering diseases (IMSEBD) has therefore been used. Immunological differences may account for the significant differences in their clinical presentation and responses to therapy, but unfortunately data on this are few. The diagnosis and management of IMSEBD on clinical grounds alone is impossible and a full history, general, and oral examination, and biopsy with immunostaining are now invariably required, sometimes supplemented with other investigations. No single treatment regimen reliably controls all these disorders, and it is not known if the specific subsets of MMP will respond to different drugs. Currently, apart from improving oral hygiene, immunomodulatory-especially immunosuppressive-therapy is typically used to control oral lesions. The present paper reviews pemphigoid, describing the present understanding of this fascinating clinical phenotype, summarising the increasing number of subsets with sometimes-different natural histories and immunological features, and outlining current clinical practice.
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Affiliation(s)
- J Bagan
- University of Valencia, Spain
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22
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Talhari C, Mahnke N, Ruzicka T, Megahed M. Successful treatment of linear IgA disease with mycophenolate mofetil as a corticosteroid sparing agent. Clin Exp Dermatol 2005; 30:297-8. [PMID: 15807696 DOI: 10.1111/j.1365-2230.2005.01741.x] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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Sánchez AR, Rogers RS, Kupp LI, Sheridan PJ. Desquamative Gingivitis Associated With IgG/IgA Pemphigoid Presents a Challenging Diagnosis and Treatment: A Case Report. J Periodontol 2004; 75:1714-9. [PMID: 15732876 DOI: 10.1902/jop.2004.75.12.1714] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
BACKGROUND Mucous membrane pemphigoid (MMP) is a heterogeneous group of autoimmune blistering disorders characterized by subepithelial separation and the deposition of immunoglobulins and complement along the basement membrane zone (BMZ). This disease is diagnosed with direct immunofluorescence testing showing a linear deposition of immunoglobulins and/or complement along the BMZ and indirect immunofluorescence testing showing circulating IgG (and sometimes IgA) autoantibodies along the BMZ. In this case report we describe desquamative gingivitis secondary to IgG/IgA pemphigoid and the management of this challenging variant of MMP. METHODS Routine histology, direct immunofluorescence testing, and indirect immunofluorescence testing were utilized and correlated to the clinical findings to diagnose this unusual immunobullous disease. RESULTS Direct and indirect immunofluorescence testing confirmed the clinical diagnosis of IgG/IgA pemphigoid as the cause of desquamative gingivitis and the other mucosal findings in this patient. A treatment program including dapsone and other drugs completely resolved the oral lesions after 14 months of therapy. CONCLUSIONS Desquamative gingivitis associated with IgG/IgA pemphigoid can be challenging to diagnose and treat. After 14 months of treatment, a combination therapy consisting of dapsone with cimetidine and vitamin E to enhance drug efficacy and frequent intramuscular administrations of triamcinolone achieved control of both the oral and genital elements of IgG/IgA pemphigoid in this patient.
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Affiliation(s)
- Andrés R Sánchez
- Division of Periodontics, Department of Dental Specialties, Mayo Clinic, Rochester, MN 55905, USA.
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Schultewolter T, Goos M, Dissemond J. Linear IgA dermatosis in an immunosuppressed patient after allogenic bone marrow transplantation. J Eur Acad Dermatol Venereol 2004; 18:721-5. [PMID: 15482308 DOI: 10.1111/j.1468-3083.2004.01065.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Linear IgA dermatosis (LAD) is a well-recognized acquired subepidermal bullous autoimmune disease. LAD is characterized by clinical, histopathological and immunopathological findings. We report the case of a 38-year-old man who suffered from a chronic myeloic leukaemia. Although he received immunosuppressive therapy he developed LAD after an allogenic bone marrow transplantation. After diagnosis of LAD was established we started a successful systemic therapy with dapsone, while continuing the preliminary medication. Here we report for the first time on a possible relationship between LAD and bone marrow transplantation in an immunosuppressed patient.
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Affiliation(s)
- T Schultewolter
- Department of Dermatology, University School of Medicine, Essen, Germany
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del Valle AE, Martínez-Sahuquillo A, Padrón JRA, Urizar JMA. Two cases of linear IgA disease with clinical manifestations limited to the gingiva. J Periodontol 2003; 74:879-82. [PMID: 12887000 DOI: 10.1902/jop.2003.74.6.879] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
Linear IgA disease (LAD) is a chronic, subepithelial blistering disease that is associated with the presence of linear deposits of IgA along the basement membrane zone. Etiopathogenic aspects of LAD are only partially known. LAD is clinically characterized by vesiculobullous skin and mucous lesions. Although more than half of LAD patients present oral mucosal lesions, there are only a few cases reported of oral lesions as the only manifestation of LAD. This study presents 2 of these uncommon cases.
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Affiliation(s)
- A Eguia del Valle
- Buccal Medicine, University of the Basque Country (EHU), Vizcaya, Spain
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26
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Kirtschig G, Murrell D, Wojnarowska F, Khumalo N. Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita. Cochrane Database Syst Rev 2003; 2003:CD004056. [PMID: 12535507 PMCID: PMC8406492 DOI: 10.1002/14651858.cd004056] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/07/2022]
Abstract
BACKGROUND Mucous membrane pemphigoid and epidermolysis bullosa acquisita are acquired autoimmune blistering diseases of the skin. Although they are rare, both can result in scarring of mucous membranes, which may lead to blindness and life threatening respiratory complications. OBJECTIVES To assess the effects of treatments for mucous membrane pemphigoid and epidermolysis bullosa acquisita. SEARCH STRATEGY Randomised Controlled Trials (RCTs) of patients with MMP or EBA were identified from MEDLINE and EMBASE from their inception to March 2000. The Cochrane Skin Group Specialised Register and the Cochrane Controlled Trials Register (CCTR) were last examined in February 2002. The bibliographies from identified studies were searched. The author who has conducted clinical trials in the field was contacted to identify unpublished trials. SELECTION CRITERIA RCTs involving participants of any ages, and with a diagnosis confirmed by immunofluorescence. Where no RCTs were located, studies with other designs were considered. DATA COLLECTION AND ANALYSIS Data were extracted from all included studies using a defined electronic data extraction protocol. Two reviewers evaluated the studies in terms of the inclusion criteria. The data from identified RCTs was extracted independently by three reviewers and subsequently checked for discrepancies. Any disagreements were resolved by discussion with each other and the fourth reviewer. Meta-analysis was not appropriate due to a lack of data. MAIN RESULTS We found two small RCTs of MMP, both conducted in patients with severe eye involvement. The same author conducted both trials. In the first trial cyclophosphamide was superior to prednisone after six months of treatment; all 12 patients responded well to cyclophosphamide versus a good response in only five of 12 patients treated with prednisone (relative risk 2.40, 95% confidence interval 1.23 to 4.69). In the second trial all 20 patients treated with cyclophosphamide responded well to it after three months of treatment, but only 14 of 20 patients responded to the treatment with dapsone (relative risk 1.4, 95% confidence interval 1.07 to 1.90). We were not able to identify a RCT of therapeutic interventions in EBA. Thirty reports of uncontrolled studies of treatment for MMP involving five or more patients and 11 reports of treatment for EBA involving two or more patients were found, but were difficult to interpret. REVIEWER'S CONCLUSIONS There is limited evidence (from two small trials) that severe ocular mucous membrane pemphigoid responds best to treatment with cyclophosphamide combined with corticosteroids, and that mild to moderate disease in most patients seems effectively suppressed by treatment with dapsone. It is difficult to make any treatment recommendations for EBA in the absence of reliable evidence sources.
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Affiliation(s)
- G Kirtschig
- Dermatology, Vrije Universiteit Medisch Centrum, PO Box 7057, Amsterdam, Netherlands, 1007 MB.
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27
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Affiliation(s)
- F Wojnarowska
- Department of Dermatology, Oxford Radcliffe Hospital, Oxford, England, United Kingdom
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Abstract
In their 60-year history, dapsone and the sulfones have been used as both antibacterial and anti-inflammatory agents. Dapsone has been used successfully to treat a range of dermatologic disorders, most successfully those characterized by abnormal neutrophil and eosinophil accumulation. This article reviews and updates the chemistry, pharmacokinetics, clinical application, mechanism of action, adverse effects, and drug interactions of dapsone and the sulfones in dermatology.
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Affiliation(s)
- Y I Zhu
- Department of Dermatology, New York Presbyterian Medical Center, 161 Fort Washington Ave., New York, NY 10032, USA
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29
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Thornhill M, Pemberton M, Buchanan J, Theaker E. An open clinical trial of sulphamethoxypyridazine in the treatment of mucous membrane pemphigoid. Br J Dermatol 2000; 143:117-26. [PMID: 10886145 DOI: 10.1046/j.1365-2133.2000.03600.x] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Twenty-five patients with mucous membrane pemphigoid (MMP), whose oral lesions were unresponsive to topical steroid treatment, were treated with 1 g daily of sulphamethoxypyridazine (SMXP), a long-acting sulphonamide antibiotic, in an open prospective clinical trial. Lesion severity was assessed objectively in a semiquantitative fashion before treatment and after 14 weeks of treatment. The patient's subjective assessment of the associated pain or discomfort, using a visual analogue scale, was also recorded at these times. Three patients (12%) were withdrawn from the study owing to side-effects or complications, one due to an allergic reaction, the other two because of significant haemolysis. For the remainder there was a significant improvement in the mean objective clinical scores for desquamative gingivitis, other oral lesions, conjunctival inflammation, nasal, vulvovaginal and skin involvement, after 14 weeks treatment with SMXP (all P < 0.001, except skin P < 0. 01). Only conjunctival scarring showed no improvement. In addition, there was a significant improvement (P < 0.001) in the pain scores for the mouth, eyes, nose, vulvovaginal region and skin. The results indicate that with appropriate monitoring SMXP is an effective treatment for MMP and compares favourably with other systemic agents used in the management of this condition.
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Affiliation(s)
- M Thornhill
- The Department of Oral Medicine, St Bartholomew's and the Royal London School of Medicine and Dentistry, Turner Street, London E1 2AD, U.K.
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Scully C, Carrozzo M, Gandolfo S, Puiatti P, Monteil R. Update on mucous membrane pemphigoid: a heterogeneous immune-mediated subepithelial blistering entity. ORAL SURGERY, ORAL MEDICINE, ORAL PATHOLOGY, ORAL RADIOLOGY, AND ENDODONTICS 1999; 88:56-68. [PMID: 10442946 DOI: 10.1016/s1079-2104(99)70194-0] [Citation(s) in RCA: 67] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Most oral involvement in the skin diseases (dermatoses) is related to mucous membrane pemphigoid or lichen planus. Mucous membrane pemphigoid was the subject of a European Symposium held in Turin, Italy, in June 1997. This review is based on that symposium. Mucous membrane pemphigoid is a subepithelial vesiculobullous disorder mainly of late middle age; it has a slight predilection for women. Whereas mucous membrane pemphigoid was formerly considered a single entity, it is now quite evident that a number of subepithelial vesiculobullous disorders may produce similar clinical pictures and also that a range of variants of mucous membrane pemphigoid exists, with antibodies directed against various hemidesmosomal components or components of the epithelial basement membrane. The term immune-mediated subepithelial blistering diseases has therefore been used. Diagnosis and management of immune-mediated subepithelial blistering diseases on clinical grounds alone are impossible; a full history, general and oral examinations, and biopsy with immunostaining are now invariably required, sometimes supplemented with other investigations. Most patients with mucous membrane pemphigoid affecting the mouth manifest desquamative gingivitis, a fairly common complaint typically seen in women who are middle-aged or older. Oral vesicles and erosions may also occur, and there can be a positive Nikolsky sign. Some patients have lesions of other stratified squamous epithelia, presenting as conjunctival, nasal, oesophageal, laryngeal, vulval, penile, or anal involvement. Apart from improving oral hygiene, immunomodulatory-in particular, immunosuppressive-therapy is typically required to control oral lesions in mucous membrane pemphigoid. No single treatment regimen reliably controls all these disorders.
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Affiliation(s)
- C Scully
- Eastman Dental Institute for Oral Health Care Science and International Centre for Excellence in Dentistry, United Kingdom.
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Kirtschig G, Mengel R, Mittag H, Flores-De-Jacoby L, Happle R. Desquamative gingivitis and balanitis--linear IgA disease or cicatricial pemphigoid? Clin Exp Dermatol 1998; 23:173-7. [PMID: 9894362 DOI: 10.1046/j.1365-2230.1998.00343.x] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
A 38-year-old man presented with gingival inflammation together with erosions of the penis. Direct immunofluorescence demonstrated linear deposits of IgA at the basement membrane zone; indirect immunofluorescence and immunoblotting were negative. Linear IgA disease (LAD) was therefore suspected and treatment with dapsone initiated but this was changed to sulfamethoxy-pyridazine and systemic corticosteroids because of methaemoglobinaemia. During 1-year follow-up the lesions continued to wax and wane although they were never as extensive as before. Eighteen months after disease onset there was scarring of the penis together with suspected fibrosis of the inflamed gingival region. In addition the patient was HLA DQ7(3) positive, a haplotype thought to be increased in patients with cicatricial pemphigoid (CP); LAD with scarring or CP with solely linear IgA deposits are possible diagnoses of his condition.
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Affiliation(s)
- G Kirtschig
- Department of Dermatology, Philipps-University Marburg, Germany
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33
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Affiliation(s)
- R J Nisengard
- School of Dental Medicine, State University of New York, Buffalo, USA
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34
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Elder MJ, Leonard J, Dart JK. Sulphapyridine--a new agent for the treatment of ocular cicatricial pemphigoid. Br J Ophthalmol 1996; 80:549-52. [PMID: 8759268 PMCID: PMC505530 DOI: 10.1136/bjo.80.6.549] [Citation(s) in RCA: 42] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
AIMS Ocular cicatricial pemphigoid (OCP) is a severe, potentially sight threatening systemic disease that sometimes requires systemic immunosuppression. This study assessed the clinical outcome of patients with OCP treated with sulphapyridine, a sulphonamide with an anti-inflammatory and immunosuppressive action but few side effects. METHODS A prospective, single armed, unmasked clinical trial was undertaken at Moorfields Eye Hospital. Twenty consecutive patients with moderate or marked conjunctival inflammation due to OCP were treated with oral sulphapyridine 500 mg twice daily. The degree of ocular inflammation was assessed as nil, mild, moderate, marked, or severe. Success was defined as resolution to mild or less. Ocular limbitis, systemic features of the disease, and side effects of the drug were also monitored. RESULTS Follow up was a mean of 12.3 (SD 4.0) months and ranged from 7 to 17 months. A successful reduction in inflammation was recorded in 22/39 eyes (56%) and 10/20 patients (50%). This improvement occurred within 1 month in 64% and in all by 2 months. Three patients developed allergy. Other side effects included nausea (n = 3), headache (n = 1), urinary hesitancy (n = 1), and mild lymphocytopenia (n = 1). These were dose dependent. Progression of cicatrisation was observed in 1/22 eyes. Success was less likely if there were systemic features of OCP or ocular limbitis. CONCLUSIONS Sulphapyridine was clinically effective in 50% of patients with moderate marked inflammation and had few side effects. It is a good alternative to dapsone.
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35
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Affiliation(s)
- J D Fine
- Department of Dermatology, University of North Carolina at Chapel Hill 27599, USA
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36
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Mallon E, Wojnarowska F. Cicatricial pemphigoid presenting with unusual palmar involvement, successfully treated with a combination of nicotinamide and tetracycline. Clin Exp Dermatol 1994; 19:526-30. [PMID: 7889682 DOI: 10.1111/j.1365-2230.1994.tb01265.x] [Citation(s) in RCA: 21] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/27/2023]
Abstract
Cicatricial pemphigoid is a rare subepidermal blistering disease, mainly of elderly women, that primarily involves mucous membranes. Oral, ocular or genital mucous membranes are most frequently involved and skin involvement is less frequent. We report a case of cicatricial pemphigoid with extensive cutaneous involvement, including unusual bilateral palmar involvement. Clinical improvement has been achieved with a combination of topical steroids, oral nicotinamide and tetracycline.
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Affiliation(s)
- E Mallon
- Department of Dermatology, Churchill Hospital, Oxford, UK
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37
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Garioch JJ, Lewis HM, Sargent SA, Leonard JN, Fry L. 25 years' experience of a gluten-free diet in the treatment of dermatitis herpetiformis. Br J Dermatol 1994; 131:541-5. [PMID: 7947207 DOI: 10.1111/j.1365-2133.1994.tb08557.x] [Citation(s) in RCA: 98] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/28/2023]
Abstract
Gluten-free diets have been used in the treatment of patients with dermatitis herpetiformis in our department since 1967. Of the 212 patients with dermatitis herpetiformis attending between 1967 and 1992, 133 managed to take the diet, and 78 of these achieved complete control of their rash by diet alone. Of the remaining 55 patients taking a gluten-free diet, all but three were taking partial diets; over half of these patients managed to substantially reduce the dose of medication required. Of the 77 patients taking a normal diet, eight entered spontaneous remission, giving a remission rate of 10%; a further two patients who had been taking gluten-free diets were found to have remitted when they resumed normal diets. Loss of IgA from the skin was observed in 10 of 41 (24%) patients taking strict gluten-free diets. These patients had been taking their diets for an average of 13 years (range 5-24 years), and their rash had been controlled by diet alone for an average of 10 years (range 3-16 years). The advantages of a gluten-free diet in the management of patients with dermatitis herpetiformis are: (i) the need for medication is reduced or abolished; (ii) there is resolution of the enteropathy, and (iii) patients experience a feeling of well-being after commencing the diet. Thus, we propose that a gluten-free diet is the most appropriate treatment for patients with dermatitis herpetiformis.
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Affiliation(s)
- J J Garioch
- Department of Dermatology, St Mary's Hospital, London, U.K
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Bernauer W, Broadway DC, Wright P. Chronic progressive conjunctival cicatrisation. Eye (Lond) 1993; 7 ( Pt 3):371-8. [PMID: 8224291 DOI: 10.1038/eye.1993.75] [Citation(s) in RCA: 29] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/29/2023] Open
Abstract
The aim of this review is to demonstrate the spectrum of conditions encompassed by the term 'chronic progressive cicatrising conjunctivitis', to discuss mechanisms of conjunctival scar tissue formation and to describe the sequelae and therapeutic options in this potentially blinding condition. Chronic progressive cicatrising conjunctivitis is found in association with some mucocutaneous disorders (cicatricial pemphigoid, linear IgA disease), as part of paraneoplastic syndromes and after long-term treatment with certain systemic and topical medications (pseudo-pemphigoid). Recent studies on the conjunctiva of pemphigoid patients indicate that macrophages may play a pivotal role in chronic progressive conjunctival cicatrisation. They mediate the transition from inflammation to scar tissue by secretion of fibrogenic cytokines. There is evidence that similar mechanisms are involved in the other fibrosing conjunctival disorders. Sequelae of chronic conjunctival cicatrisation include the obstruction of lacrimal and meibomian glands, tear film alterations, trichiasis, keratopathy and blindness. Present possibilities and future options for the treatment of this condition are discussed.
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Jabłońska S, Chorzelski TP, Rosinska D, Maciejowska E. Linear IgA bullous dermatosis of childhood (chronic bullous dermatosis of childhood). Clin Dermatol 1991; 9:393-401. [PMID: 1806227 DOI: 10.1016/0738-081x(91)90031-f] [Citation(s) in RCA: 37] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Affiliation(s)
- S Jabłońska
- Department of Dermatology, Warsaw Academy of Medicine, Poland
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40
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Affiliation(s)
- T P Chorzelski
- Department of Dermatology, Warsaw Academy of Medicine, Poland
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41
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Affiliation(s)
- J N Leonard
- Department of Dermatology, St. Mary's Hospital, London, England
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42
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Godfrey KM, Wojnarowska F, Friedland JS. Obliterative bronchiolitis and alveolitis associated with sulphamethoxypyridazine (Lederkyn) therapy for linear IgA disease of adults. Br J Dermatol 1990; 123:125-6. [PMID: 2202426 DOI: 10.1111/j.1365-2133.1990.tb01833.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
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