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Chi LQ, Anh NTV, Le NNQ, Ha NTT, Tien HM, Dien TM. Successful Treatment of Carbamazepine-Induced Toxic Epidermal Necrolysis With Clinical Gastrointestinal Involvement: A Case Report. Front Pediatr 2022; 10:834037. [PMID: 35498784 PMCID: PMC9043682 DOI: 10.3389/fped.2022.834037] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/12/2021] [Accepted: 03/14/2022] [Indexed: 12/17/2022] Open
Abstract
BACKGROUND Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare and life-threatening disease of the skin and mucosal surfaces. Although gastrointestinal manifestations in adults are potential prognostic factors for disease severity, there are limited data on such cases and their standard management in the pediatric population. CASE PRESENTATION We herein report the case of an 8-year-old girl with a 1-year history of epilepsy, who presented with bilateral conjunctivitis and progressively widespread bullous, and pruritic eruption based on erythematous skin after administration of carbamazepine. A diagnosis of carbamazepine-induced TEN was made, and the drug was immediately discontinued. The result of genetic screening showed that the patient was positive for the HLA-B*15:02 allele. Then, her condition got worse by developing gastrointestinal involvement, including hematemesis and severe watery bloody diarrhea. A combination of the intravenous immunoglobulin and the appropriate dose of systemic steroids have contributed to a favorable outcome in this case. Multidisciplinary care of mucocutaneous involvement, supplemental nutrition, and fluid replacement was also critically warranted. This report aims to contribute to the current literature on TEN-related gastrointestinal manifestations in pediatrics and highlights the need for further investigations in determining the optimal treatment in such cases. CONCLUSION In conclusion, we reported the successful treatment of TEN-related gastrointestinal manifestations in a pediatric patient, which should be critically considered in patients with SJS/TEN. Since it may significantly contribute to the poor prognosis of the illness, further investigations in determining standard management in such cases are necessary.
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Affiliation(s)
- Le Quynh Chi
- Department of Rheumatology, Allergy, and Immunology, Vietnam National Children's Hospital, Hanoi, Vietnam
| | - Nguyen Thi Van Anh
- Department of Rheumatology, Allergy, and Immunology, Vietnam National Children's Hospital, Hanoi, Vietnam
| | - Nguyen Ngoc Quynh Le
- Department of Rheumatology, Allergy, and Immunology, Vietnam National Children's Hospital, Hanoi, Vietnam
| | - Nguyen Thi Thu Ha
- Department of Rheumatology, Allergy, and Immunology, Vietnam National Children's Hospital, Hanoi, Vietnam
| | - Hoang Minh Tien
- Department of Rheumatology, Allergy, and Immunology, Vietnam National Children's Hospital, Hanoi, Vietnam
| | - Tran Minh Dien
- Surgical Intensive Care Unit, Vietnam National Children's Hospital, Hanoi, Vietnam
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Bechek S, Garcia M, Chiou H. Severe Gastrointestinal Involvement in Pediatric Stevens-Johnson Syndrome: A Case Report and Review of the Literature. Clin Exp Gastroenterol 2020; 13:377-383. [PMID: 33061516 PMCID: PMC7533238 DOI: 10.2147/ceg.s269349] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/25/2020] [Accepted: 08/11/2020] [Indexed: 12/11/2022] Open
Abstract
Stevens-Johnson syndrome and toxic epidermal necrolysis form a rare but severe disease spectrum characterized by widespread epidermal detachment. Gastrointestinal manifestations of the disease, however, are rarely described in the pediatric literature and have a high mortality among adults. There are limited data on the treatment of these cases, with conflicting evidence regarding the benefit of steroids, IVIG, or other immunosuppressive agents. We review previous instances of gastrointestinal involvement in children and report the case of a previously healthy 13-year-old who presented with the typical ocular and skin findings of Stevens-Johnson syndrome, subsequently developed severe life-threatening diarrhea, and was found to have severe esophagitis, duodenitis, and colitis on endoscopic evaluation. Treatment was initiated with an immediate, short course of steroids along with early introduction of an enteral diet via nasogastric tube, and resulted in full gastrointestinal recovery. This case highlights successful medical treatment of the first reported pediatric case of SJS/TEN with both upper and lower gastrointestinal tract involvement.
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Affiliation(s)
- Sophia Bechek
- Stanford University School of Medicine, Stanford, CA, USA
| | - Manuel Garcia
- Division of Pediatric Gastroenterology, Stanford University School of Medicine, Stanford, CA, USA
| | - Howard Chiou
- Department of Medicine, Santa Clara Valley Medical Center, San Jose, CA, USA
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Kaide CG, San Miguel CE. Stevens Johnson Syndrome – “Steven Who? And Why I Should Care About His Johnson?”. CASE STUDIES IN EMERGENCY MEDICINE 2020. [PMCID: PMC7122494 DOI: 10.1007/978-3-030-22445-5_55] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Abstract
Diagnosing rashes in the emergency department can be challenging. If the rash is extensive and targetoid, include Stevens-Johnson Syndrome (SJS) on your differential and be sure to obtain a thorough history to identify possible offending agents. Additionally, prioritize supportive care and obtaining a dermatology/burn consult to coordinate appropriate care.
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Affiliation(s)
- Colin G. Kaide
- Wexner Medical Center, Ohio State University, Columbus, OH USA
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Colonic Involvement of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Rare Cause of Gastrointestinal Bleeding. ACG Case Rep J 2019; 6:e00242. [PMID: 31832469 PMCID: PMC6855542 DOI: 10.14309/crj.0000000000000242] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/11/2019] [Accepted: 09/11/2019] [Indexed: 01/18/2023] Open
Abstract
Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) represents a spectrum of rare but severe mucocutaneous drug reactions. Gastrointestinal involvement of SJS/TEN is associated with high morbidity and mortality and often presents 2-3 weeks after the initial appearance of skin lesions. There are no evidence-based treatment algorithms for the management of SJS/TEN. We report a case of life-threatening gastrointestinal bleeding from colonic involvement of SJS/TEN and discuss potential therapeutic options.
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Abstract
Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are immunologic reactions to several stimuli, mostly medications, which present as a spectrum of primarily widespread mucocutaneous lesions, but also with other organ involvement. Pathology is characterized by full thickness necrosis of the epithelial layer of the involved organ due to immune-mediated apoptosis of the resident keratinocytes. High suspicion for early detection and quick withdrawal of the culprit medication are the most important steps in stopping this reaction. Aggressive supportive care is often necessary as the patient recovers. Steroids, other immunosuppressants, and plasmapheresis have all been studied as treatments, but high-quality evidence supporting their contributions, either together or separately, in decreasing length of hospital stay or prolonging survival have not been consistently demonstrated. Further studies of the mechanism of action and novel treatment modalities are still needed to improve outcomes in patients with this rare but often fatal condition.
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Affiliation(s)
- Joseph L. Nates
- Department of Critical Care and Respiratory Care, The University of Texas MD Anderson Cancer Center, Houston, TX USA
| | - Kristen J. Price
- Division of Anesthesiology, Critical Care and Pain Medicine, Department of Critical Care and Respiratory Care, The University of Texas MD Anderson Cancer Center, Houston, TX USA
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Jha AK, Suchismita A, Jha RK, Raj VK. Spectrum of gastrointestinal involvement in Stevens - Johnson syndrome. World J Gastrointest Endosc 2019; 11:115-123. [PMID: 30788030 PMCID: PMC6379748 DOI: 10.4253/wjge.v11.i2.115] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/17/2018] [Revised: 01/29/2019] [Accepted: 02/13/2019] [Indexed: 02/06/2023] Open
Abstract
Stevens - Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) is a severe adverse drug reaction associated with involvement of skin and mucosal membranes, and carries significant risk of mortality and morbidity. Mucus membrane lesions usually involve the oral cavity, lips, bulbar conjunctiva and the anogenitalia. The oral/anal mucosa and liver are commonly involved in SJS or TEN. However, intestinal involvement is distinctly rare. We herein review the current literature regarding the gastrointestinal involvement in SJS or TEN. This review focuses mainly on the small bowel and colonic involvement in patients with SJS or TEN.
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Affiliation(s)
- Ashish Kumar Jha
- Department of Gastroenterology, Indira Gandhi Institute of Medical Science, Sheikhpura, Patna 800014, India
| | - Arya Suchismita
- Department of Pediatrics, Indira Gandhi Institute of Medical Science, Sheikhpura, Patna 800014, India
| | | | - Vikas Kumar Raj
- Health Center, National Institute of Technology, Patna 800014, India
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Abstract
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered a delayed-type hypersensitivity reaction to drugs. They represent true medical emergencies and an early recognition and appropriate management is decisive for the survival. SJS/TEN manifest with an "influenza-like" prodromal phase (malaise, fever), followed by painful cutaneous and mucous membrane (ocular, oral, and genital) lesions, and other systemic symptoms. The difference between SJS, SJS/TEN overlap, and TEN is defined by the degree of skin detachment: SJS is defined as skin involvement of < 10%, TEN is defined as skin involvement of > 30%, and SJS/TEN overlap as 10-30% skin involvement. The diagnosis of different degrees of epidermal necrolysis is based on the clinical assessment in conjunction with the corresponding histopathology. The mortality rates for SJS and TEN have decreased in the last decades. Today, the severity-of-illness score for toxic epidermal necrolysis (SCORTEN) is available for SJS/TEN severity assessment. Drugs with a high risk of causing SJS/TEN are anti-infective sulfonamides, anti-epileptic drugs, non-steroidal anti-inflammatory drugs of the oxicam type, allopurinol, nevirapine, and chlormezanone. Besides conventional drugs, herbal remedies and new biologicals should be considered as causative agents. The increased risk of hypersensitivity reactions to certain drugs may be linked to specific HLA antigens. Our understanding of the pathogenesis of SJS/TEN has improved: drug-specific T cell-mediated cytotoxicity, genetic linkage with HLA- and non-HLA-genes, TCR restriction, and cytotoxicity mechanisms were clarified. However, many factors contributing to epidermal necrolysis still have to be identified, especially in virus-induced and autoimmune forms of epidermal necrolysis not related to drugs. In SJS/TEN, the most common complications are ocular, cutaneous, or renal. Nasopharyngeal, esophageal, and genital mucosal involvement with blisters, erosions as well as secondary development of strictures also play a role. However, in the acute phase, septicemia is a leading cause of morbidity and fatality. Pulmonary and hepatic involvement is frequent. The acute management of SJS/TEN requires a multidisciplinary approach. Immediate withdrawal of potentially causative drugs is mandatory. Prompt referral to an appropriate medical center for specific supportive treatment is of utmost importance. The most frequently used treatments for SJS/TEN are systemic corticosteroids, immunoglobulins, and cyclosporine A.
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Peter JG, Lehloenya R, Dlamini S, Risma K, White KD, Konvinse KC, Phillips EJ. Severe Delayed Cutaneous and Systemic Reactions to Drugs: A Global Perspective on the Science and Art of Current Practice. THE JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY. IN PRACTICE 2017; 5:547-563. [PMID: 28483310 PMCID: PMC5424615 DOI: 10.1016/j.jaip.2017.01.025] [Citation(s) in RCA: 103] [Impact Index Per Article: 12.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/24/2016] [Revised: 01/11/2017] [Accepted: 01/18/2017] [Indexed: 12/17/2022]
Abstract
Most immune-mediated adverse drug reactions (IM-ADRs) involve the skin, and many have additional systemic features. Severe cutaneous adverse drug reactions (SCARs) are an uncommon, potentially life-threatening, and challenging subgroup of IM-ADRs with diverse clinical phenotypes, mechanisms, and offending drugs. T-cell-mediated immunopathology is central to these severe delayed reactions, but effector cells and cytokines differ by clinical phenotype. Strong HLA-gene associations have been elucidated for specific drug-SCAR IM-ADRs such as Stevens-Johnson syndrome/toxic epidermal necrolysis, although the mechanisms by which carriage of a specific HLA allele is necessary but not sufficient for the development of many IM-ADRs is still being defined. SCAR management is complicated by substantial short- and long-term morbidity/mortality and the potential need to treat ongoing comorbid disease with related medications. Multidisciplinary specialist teams at experienced units should care for patients. In the setting of SCAR, patient outcomes as well as preventive, diagnostic, treatment, and management approaches are often not generalizable, but rather context specific, driven by population HLA-genetics, the pharmacology and genetic risk factors of the implicated drug, severity of underlying comorbid disease necessitating ongoing treatments, and cost considerations. In this review, we update the basic and clinical science of SCAR diagnosis and management.
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Affiliation(s)
- Jonathan Grant Peter
- Division of Allergology and Clinical Immunology, Department of Medicine, University of Cape Town, Cape Town, South Africa
| | - Rannakoe Lehloenya
- Division of Dermatology, Department of Medicine, University of Cape Town, Cape Town, South Africa
| | - Sipho Dlamini
- Division of Infectious Diseases, Department of Medicine, University of Cape Town, Cape Town, South Africa
| | - Kimberly Risma
- Division of Allergy, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio
| | - Katie D White
- Department of Medicine, Vanderbilt University Medical Center, Nashville, Tenn
| | - Katherine C Konvinse
- Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, Tenn
| | - Elizabeth J Phillips
- Department of Medicine, Vanderbilt University Medical Center, Nashville, Tenn; Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, Tenn; Department of Pharmacology, Vanderbilt University School of Medicine, Nashville, Tenn; Institute for Immunology & Infectious Diseases, Murdoch University, Murdoch, Western Australia, Australia.
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Saeed H, Mantagos IS, Chodosh J. Complications of Stevens–Johnson syndrome beyond the eye and skin. Burns 2016; 42:20-27. [DOI: 10.1016/j.burns.2015.03.012] [Citation(s) in RCA: 39] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/26/2014] [Revised: 03/01/2015] [Accepted: 03/20/2015] [Indexed: 11/27/2022]
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Mesenteric ischemia secondary to toxic epidermal necrolysis: case report and review of the literature. J Burn Care Res 2015; 35:e346-52. [PMID: 24496304 DOI: 10.1097/bcr.0000000000000006] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
A 28-year-old otherwise healthy man was admitted to the burn center for treatment of toxic epidermal necrolysis (TEN) involving 90% of the TBSA and oropharynx. On hospital day 8, his cutaneous lesions were healing well, but he developed respiratory distress, fever, and abdominal distension. Computerized tomography demonstrated distended bowel, pneumatosis intestinalis, and portal venous gas. He underwent emergent celiotomy. Patchy areas of nonperforated necrosis along the jejunum and ileum were present. No mechanical or embolic source of ischemia could be identified. A 120-cm segment of ischemic small bowel was resected and the abdomen was closed temporarily. On planned "second look" the following day, no further disease was encountered and intestinal continuity was restored. Tube feeds were then initiated and the patient's recovery was uneventful thereafter. Although traditionally considered a skin disorder, TEN may be more accurately described as a disorder affecting the junction of an epithelium and its supporting tissue. It is most prominently manifested at the epidermal-dermal junction, but epithelial-submucosal junctions are also affected. The ocular, respiratory, genitourinary, and gastrointestinal manifestations of TEN are variable and incompletely understood. This disease is rooted in immunological dysfunction and the small bowel is rich in immunologically active tissue; Peyer patches and lymph nodes abound. Clinicians should be vigilant for gastrointestinal tract involvement, which is potentially treatable with resection of the ischemic bowel. The authors suspect that, given the critical condition of many TEN patients, bowel symptoms may be incorrectly attributed to global hypoperfusion and sepsis.
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Fava P, Astrua C, Cavaliere G, Brizio M, Savoia P, Quaglino P, Fierro MT. Intestinal involvement in toxic epidermal necrolysis. A case report and review of literature. J Eur Acad Dermatol Venereol 2014; 29:1843-5. [PMID: 24754517 DOI: 10.1111/jdv.12535] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Affiliation(s)
- P Fava
- Department of Medical Sciences, Dermatologic Clinic, University of Turin, Torino, Italy
| | - C Astrua
- Department of Medical Sciences, Dermatologic Clinic, University of Turin, Torino, Italy
| | - G Cavaliere
- Department of Medical Sciences, Dermatologic Clinic, University of Turin, Torino, Italy
| | - M Brizio
- Department of Medical Sciences, Dermatologic Clinic, University of Turin, Torino, Italy
| | - P Savoia
- Department of Medical Sciences, Dermatologic Clinic, University of Turin, Torino, Italy
| | - P Quaglino
- Department of Medical Sciences, Dermatologic Clinic, University of Turin, Torino, Italy
| | - M T Fierro
- Department of Medical Sciences, Dermatologic Clinic, University of Turin, Torino, Italy
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Heye P, Descloux A, Singer G, Rosenberg R, Kocher T. Perforated sigmoid diverticulitis in the presence of toxic epidermal necrolysis. Case Rep Dermatol 2014; 6:49-53. [PMID: 24707250 PMCID: PMC3975207 DOI: 10.1159/000360129] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022] Open
Abstract
Even though the incidence of toxic epidermal necrolysis (TEN) is low, it is also associated with a high mortality rate. The condition predominantly affects the skin, but may also affect the gastrointestinal tract, dramatically increasing mortality. We present a case of perforated sigmoid diverticulitis in the presence of TEN. The patient was taking medication, known to be a risk factor, and presented an affected total body surface area and temporal development similar to previously reported cases of TEN. Characteristic abdominal symptoms, however, were missing. Gastrointestinal involvement in TEN appears to be a poor prognostic factor; medical staff must therefore be alert to patients with TEN who complain of abdominal discomfort. The exact pathogenesis, however, remains unclear.
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Affiliation(s)
- P Heye
- Department of Surgery, Kantonsspital Baden, Baden, Switzerland
| | - A Descloux
- Department of Surgery, Kantonsspital Baden, Baden, Switzerland
| | - G Singer
- Department of Pathology, Kantonsspital Baden, Baden, Switzerland
| | - R Rosenberg
- Department of Surgery, Kantonsspital Baden, Baden, Switzerland
| | - T Kocher
- Department of Surgery, Kantonsspital Baden, Baden, Switzerland
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The First Reported Case of Ureteral Perforation in a Patient With Severe Toxic Epidermal Necrolysis Syndrome. J Burn Care Res 2014; 35:e265-8. [DOI: 10.1097/bcr.0b013e31829a4374] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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Abstract
OBJECTIVES The aims of this review are to summarize the definitions, causes, and clinical course as well as the current understanding of the genetic background, mechanism of disease, and therapy of toxic epidermal necrolysis and Stevens-Johnson syndrome. DATA SOURCES PubMed was searched using the terms toxic epidermal necrolysis, Stevens-Johnson syndrome, drug toxicity, drug interaction, and skin diseases. DATA SYNTHESIS Toxic epidermal necrolysis and Stevens-Johnson syndrome are acute inflammatory skin reactions. The onset is usually triggered by infections of the upper respiratory tract or by preceding medication, among which nonsteroidal anti-inflammatory agents, antibiotics, and anticonvulsants are the most common triggers. Initially the diseases present with unspecific symptoms, followed by more or less extensive blistering and shedding of the skin. Complete death of the epidermis leads to sloughing similar to that seen in large burns. Toxic epidermal necrolysis is the most severe form of drug-induced skin reaction and includes denudation of >30% of total body surface area. Stevens-Johnson syndrome affects <10%, whereas involvement of 10%-30% of body surface area is called Stevens-Johnson syndrome/toxic epidermal necrolysis overlap. Besides the skin, mucous membranes such as oral, genital, anal, nasal, and conjunctival mucosa are frequently involved in toxic epidermal necrolysis and Stevens-Johnson syndrome. Toxic epidermal necrolysis is associated with a significant mortality of 30%-50% and long-term sequelae. Treatment includes early admission to a burn unit, where treatment with precise fluid, electrolyte, protein, and energy supplementation, moderate mechanical ventilation, and expert wound care can be provided. Specific treatment with immunosuppressive drugs or immunoglobulins did not show an improved outcome in most studies and remains controversial. The mechanism of disease is not completely understood, but immunologic mechanisms, cytotoxic reactions, and delayed hypersensitivity seem to be involved. CONCLUSION Profound knowledge of exfoliative skin diseases is needed to improve therapy and outcome of these life-threatening illnesses.
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Bouziri A, Khaldi A, Hamdi A, Borgi A, Ghorbel S, Kharfi M, Hadj SB, Menif K, Ben Jaballah N. Toxic epidermal necrolysis complicated by small bowel intussusception: a case report. J Pediatr Surg 2011; 46:e9-e11. [PMID: 21292071 DOI: 10.1016/j.jpedsurg.2010.09.011] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/14/2010] [Revised: 08/30/2010] [Accepted: 09/01/2010] [Indexed: 12/19/2022]
Abstract
Intestinal involvement in toxic epidermal necrolysis (TEN) has been identified only rarely. We report a case of TEN complicated by small bowel intussusception. The patient was a previously healthy 8-year-old boy who presented with TEN and extensive lesions, including up to 40% of the body surface area as well as conjunctival, oropharyngeal, respiratory, and genital mucosa. Rapidly after the onset of a constant rate of enteral feeding, he developed bilious vomiting, diarrhea, and significant abdominal distension. Abdominal sonography showed a small bowel intussusception. At abdominal exploration, an ileoileal intussusception was observed with a viable but inflamed bowel wall. Manual reduction was performed. During the postoperative clinical course, the patient was managed with total parenteral nutrition and local care of the skin and mucous membranes. Enteral feeding was introduced on the sixth postoperative day, and the child left the hospital 15 days after his admission. The association of TEN and small bowel intussusception has not been previously reported in the literature.
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Affiliation(s)
- Asma Bouziri
- Paediatric Intensive Care Unit, Children's Hospital of Tunis, Tunis, Tunisia.
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Kedward AL, McKenna K. A fatal case of toxic epidermal necrolysis with extensive intestinal involvement. Clin Exp Dermatol 2010; 34:e484. [PMID: 19747323 DOI: 10.1111/j.1365-2230.2009.03553.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
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Sakai N, Yoshizawa Y, Amano A, Higashi N, Aoki M, Seo T, Suzuki K, Tanaka S, Tsukui T, Sakamoto C, Arai M, Yamamoto Y, Kawana S. Toxic epidermal necrolysis complicated by multiple intestinal ulcers. Int J Dermatol 2008; 47:180-2. [PMID: 18211494 DOI: 10.1111/j.1365-4632.2008.03389.x] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Affiliation(s)
- Noriyasu Sakai
- Departments of Dermatology and Digestive Medicine, and Center of Emergency Medicine, Nippon Medical School, Tokyo, Japan.
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Al-Johani KA, Fedele S, Porter SR. Erythema multiforme and related disorders. ACTA ACUST UNITED AC 2007; 103:642-54. [PMID: 17344075 DOI: 10.1016/j.tripleo.2006.12.008] [Citation(s) in RCA: 55] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/27/2006] [Revised: 12/12/2006] [Accepted: 12/12/2006] [Indexed: 01/07/2023]
Abstract
Erythema multiforme (EM) and related disorders comprise a group of mucocutaneous disorders characterized by variable degrees of mucosal and cutaneous blistering and ulceration that occasionally can give rise to systemic upset and possibly compromise life. The clinical classification of these disorders has often been variable, thus making definitive diagnosis sometimes difficult. Despite being often caused by, or at least associated with, infection or drug therapy, the pathogenic mechanisms of these disorders remain unclear, and as a consequence, there are no evidence-based, reliably effective therapies. The present article reviews aspects of EM and related disorders of relevance to oral medicine clinical practice and highlights the associated potential etiologic agents, pathogenic mechanisms and therapies.
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Affiliation(s)
- Khalid A Al-Johani
- Division of Medical, Surgical and Diagnostic Sciences, Eastman Dental Institute for Oral Health Care Sciences, University College of London, London, England
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Abstract
Toxic epidermal necrolysis (TEN) is a rare but serious condition with a high mortality rate, whose clinical features resemble major thermal injury. Nutritional substrates which can affect the immune response have been shown to benefit burned and critically ill patients but there is a lack of data in the TEN literature. A case study is presented of a 63-year-old female admitted to our burn unit with extensive skin and mucosal involvement. Immune modulating artificial nutrition support was given initially via the parenteral route and then the nasogastric route. Maintenance of the patient's nutritional status and clinical outcome suggest that there may be a role for this type of intervention in TEN.
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Affiliation(s)
- E M Windle
- Department of Nutrition and Dietetics, Pinderfields General Hospital, Wakefield, UK.
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