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Bordeanu-Diaconescu EM, Grama S, Grosu-Bularda A, Frunză A, Dumitru CŞ, Andrei MC, Creangă CA, Neagu TP, Lascăr I. Toxic epidermal necrolysis - clinicopathological aspects and therapeutic management. ROMANIAN JOURNAL OF MORPHOLOGY AND EMBRYOLOGY = REVUE ROUMAINE DE MORPHOLOGIE ET EMBRYOLOGIE 2024; 65:765-773. [PMID: 39957038 PMCID: PMC11924910 DOI: 10.47162/rjme.65.4.23] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/31/2024] [Accepted: 01/03/2025] [Indexed: 02/18/2025]
Abstract
Toxic epidermal necrolysis (TEN) is a serious dermatological condition often triggered by different drugs or medications or, less commonly, by infections, leading to extensive epidermal detachment and multisystemic complications, resembling the severity and systemic impact of burn injuries. This case report portrays a 26-year-old female patient with a history of psychiatric treatment and recreational drug use, presenting with typical prodromal symptoms and characteristic manifestations on the integument and mucosae. Clinical management involved an interdisciplinary team in a burn center, administering immunoglobulins, systemic steroids, and supportive therapies to prevent complications including infection and to support skin re-epithelization. Histopathological findings confirmed the diagnosis. Despite the extensive lesions, prompt treatment facilitated a positive outcome. The report emphasizes the necessity of referral to specialized centers and the complex, multidisciplinary management required for TEN patients to optimize survival and minimize long-term sequelae.
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Affiliation(s)
- Eliza Maria Bordeanu-Diaconescu
- Discipline of Plastic and Reconstructive Surgery, Department 11, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania;
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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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Gao X, Tang X, Ai L, Gao Q, Liao Q, Chen M, Chen X, Zhou H, Ye Y, Li M, Han J, Wang F. Acute pancreatic injuries: A complication of Stevens-Johnson syndrome/toxic epidermal necrolysis associated with cytotoxic immunocell activation. J Am Acad Dermatol 2020; 84:644-653. [PMID: 32561372 PMCID: PMC7297678 DOI: 10.1016/j.jaad.2020.06.043] [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] [Subscribe] [Scholar Register] [Received: 04/01/2020] [Revised: 06/07/2020] [Accepted: 06/10/2020] [Indexed: 10/25/2022]
Abstract
BACKGROUND Complications involving internal organs are usually present in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, pancreatic complications are rarely reported and studied. OBJECTIVE To summarize clinical characteristics of SJS/TEN-associated acute pancreatic injuries and to investigate underlying inflammatory mechanisms. METHODS Clinical records of 124 inpatients with SJS/TEN were reviewed. Serum levels of tumor necrosis factor α, interleukin (IL) 6, IL-18, IL-15, IL-12p70, and soluble CD56 were determined in 18 healthy donors and 17 patients with SJS/TEN, including 3 with acute pancreatic injuries. RESULTS Acute pancreatic injury was diagnosed in 7.3% of patients (9/124) in the SJS/TEN cohort. Elevation of serum transaminase level and hypoalbuminemia occurred more frequently in patients with acute pancreatic injuries compared with those without pancreatic symptoms (P = .004 and <.001, respectively). Although acute pancreatic injury did not alter mortality rate of SJS/TEN, it was associated with longer hospitalization stays (P = .008). Within the serum cytokines whose levels were elevated in SJS/TEN, only IL-18 was found to be selectively increased in patients with acute pancreatic injuries compared with those without them (P = .03). LIMITATIONS Cohort was small. CONCLUSION Acute pancreatic injury is a gastrointestinal complication of SJS/TEN in which hepatotoxicity is more likely to occur. Overexpression of IL-18 might be involved in this unique entity.
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Affiliation(s)
- Xuemei Gao
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong
| | - Xuhua Tang
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong
| | - Lu Ai
- Department of Laboratory Medicine, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong
| | - Qian Gao
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong
| | - Qiman Liao
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong
| | - Mukai Chen
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong
| | - Xiaohong Chen
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong
| | - Hui Zhou
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong
| | - Yanting Ye
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong
| | - Minyi Li
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong
| | - Jiande Han
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong.
| | - Fang Wang
- Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong.
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Kvarme H, Fjellanger R, Bondevik C, Astor MC, Barrett T, Haugen O, Storebråten G, Karliczek A, Vaagbø G, Sande Leikanger I, Tveit KS, Engjom T, von Volkmann HL. A young male with epitheliolysis in the small bowel and colon. Scand J Gastroenterol 2020; 55:631-633. [PMID: 32393134 DOI: 10.1080/00365521.2020.1760344] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Background: Toxic epidermal necrolysis (TEN) is characterized by epidermal necrosis of various degree, and can affect the entire body surface. Affection of small bowel and colon is a rare manifestation of TEN. We present a case with an unusual appearance of epitheliolysis of the small bowel and colon due to a toxic reaction.Case report: A 19 year old male was diagnosed with ulcerative colitis (UC) after treatment with tetracyclines followed by isotretinoin due to acne vulgaris. Medical treatment did not lead to improvement of his UC, and an emergency resection of the colon was performed. Postoperatively his condition worsened due to small bowel epitheliolysis, and he recovered finally 6 months later after a partial small bowel resection.Conclusion: The true cause of this very serious situation with severe gastrointestinal involvement is not fully understood. In this case, successive treatment with antibiotics and isotretinoin given to a patient with an inflamed colon might have triggered the destruction of the epithelial barrier, leading to an immense immunological reaction in the intestinal wall. We suggest that physicians should be aware of UC-like symptoms occurring prior to or during treatment with tetracyclines and/or isotretinoin.
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Affiliation(s)
- Heidi Kvarme
- Department of Medicine, Haukeland University Hospital, Bergen, Norway
| | - Renée Fjellanger
- Department of Medicine, Haukeland University Hospital, Bergen, Norway
| | - Camilla Bondevik
- Department of Medicine, Haukeland University Hospital, Bergen, Norway
| | | | - Torill Barrett
- Department of Pathology, Haukeland University Hospital, Bergen, Norway
| | - Oddbjørn Haugen
- Department of anesthesiology, Intensive Care Unit, Haukeland University Hospital, Bergen, Norway
| | - Gøril Storebråten
- Department of Surgery, Haukeland University Hospital, Bergen, Norway
| | - Anne Karliczek
- Department of Surgery, Haukeland University Hospital, Bergen, Norway
| | - Guro Vaagbø
- Department of Occupational Medicine, Hyperbaric Medical Unit, Haukeland University Hospital, Bergen, Norway
| | | | - Kåre Steinar Tveit
- Department of Dermatology, Haukeland University Hospital, Bergen, Norway
| | - Trond Engjom
- Department of Medicine, Haukeland University Hospital, Bergen, Norway
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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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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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