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著者: Yung-Tsu Cho, Jheng-Wei Lin, Yi-Chun Chen, Chia-Ying Chang, Cheng-Hsiang Hsiao, Wen-Hung Chung, Chia-Yu Chu
雑誌名: J Am Acad Dermatol. 2014 Mar;70(3):539-48. doi: 10.1016/j.jaad.2013.11.015. Epub 2014 Jan 2.
Abstract/Text
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.
Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.
PMID 24388722 J Am Acad Dermatol. 2014 Mar;70(3):539-48. doi: 10.1016/j.jaad.2013.11.015. Epub 2014 Jan 2.
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