Yung-Tsu Cho1, Jheng-Wei Lin2, Yi-Chun Chen3, Chia-Ying Chang1, Cheng-Hsiang Hsiao4, Wen-Hung Chung2, Chia-Yu Chu5. 1. Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan. 2. Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Keelung and Linkou Branches, College of Medicine, Chang Gung University, Linkou, Taiwan. 3. Department of Dermatology, Cathay General Hospital, Taipei, Taiwan. 4. Department of Pathology, Cheng Shin General Hospital, Taipei, Taiwan. 5. Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan. Electronic address: chiayu@ntu.edu.tw.
Abstract
BACKGROUND: Generalized bullous fixed drug eruption (GBFDE), a particular form of fixed drug eruption (FDE), is characterized by widespread blisters and erosions and can be confused with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). OBJECTIVE: We sought to analyze specific features of GBFDE and differentiate it from SJS/TEN. METHODS: We retrospectively studied patients with GBFDE and SJS/TEN during a period of 10 years. GBFDE was defined as typical FDE lesions with blisters involving at least 10% body surface area on at least 3 of 6 different anatomic sites. Clinical presentations; histopathological features; immunohistochemical patterns of cluster-of-differentiation (CD)3, CD4, CD8, CD56, Fas, Fas ligand, granzyme B, perforin, granulysin, and forkhead box P3 (Foxp3); and serum granulysin levels were compared. RESULTS: Twenty-three cases of GBFDE were collected. Patients with GBFDE had shorter latent periods, less mucosal involvement, more eosinophil infiltration, and dermal melanophages. Lesional infiltrates in GBFDE had more dermal CD4(+) cells including Foxp3(+) regulatory T cells, fewer intraepidermal CD56(+) cells, and fewer intraepidermal granulysin(+) cells. The serum level of granulysin in GBFDE was also significantly lower than in SJS/TEN. LIMITATIONS: The number of cases in this study is small. CONCLUSION: GBFDE is a distinct disease distinguishable from SJS/TEN by particular features such as granulysin, CD56, and Foxp3 expressions.
BACKGROUND: Generalized bullous fixed drug eruption (GBFDE), a particular form of fixed drug eruption (FDE), is characterized by widespread blisters and erosions and can be confused with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). OBJECTIVE: We sought to analyze specific features of GBFDE and differentiate it from SJS/TEN. METHODS: We retrospectively studied patients with GBFDE and SJS/TEN during a period of 10 years. GBFDE was defined as typical FDE lesions with blisters involving at least 10% body surface area on at least 3 of 6 different anatomic sites. Clinical presentations; histopathological features; immunohistochemical patterns of cluster-of-differentiation (CD)3, CD4, CD8, CD56, Fas, Fas ligand, granzyme B, perforin, granulysin, and forkhead box P3 (Foxp3); and serum granulysin levels were compared. RESULTS: Twenty-three cases of GBFDE were collected. Patients with GBFDE had shorter latent periods, less mucosal involvement, more eosinophil infiltration, and dermal melanophages. Lesional infiltrates in GBFDE had more dermal CD4(+) cells including Foxp3(+) regulatory T cells, fewer intraepidermal CD56(+) cells, and fewer intraepidermal granulysin(+) cells. The serum level of granulysin in GBFDE was also significantly lower than in SJS/TEN. LIMITATIONS: The number of cases in this study is small. CONCLUSION: GBFDE is a distinct disease distinguishable from SJS/TEN by particular features such as granulysin, CD56, and Foxp3 expressions.
Authors: Jonathan Grant Peter; Rannakoe Lehloenya; Sipho Dlamini; Kimberly Risma; Katie D White; Katherine C Konvinse; Elizabeth J Phillips Journal: J Allergy Clin Immunol Pract Date: 2017 May - Jun