YUNG-TSU CHOLin, Jheng-WeiJheng-WeiLinChen, Yi-ChunYi-ChunChenChang, Chia-YingChia-YingChangHsiao, Cheng-HsiangCheng-HsiangHsiaoChung, Wen-HungWen-HungChungCHIA-YU CHU2019-12-042019-12-042014-030190-9622https://www.scopus.com/inward/record.uri?eid=2-s2.0-84894057183&doi=10.1016%2fj.jaad.2013.11.015&partnerID=40&md5=4ac8a21b05c89910ab5482c60bc605cbhttps://scholars.lib.ntu.edu.tw/handle/123456789/434907Background 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. ? 2013 by the American Academy of Dermatology, Inc.enfixed drug eruptiongeneralized bullous fixed drug eruptiongranulysinregulatory T cellsStevens-Johnson syndrometoxic epidermal necrolysis[SDGs]SDG3fixed drug eruption; generalized bullous fixed drug eruption; granulysin; regulatory T cells; Stevens-Johnson syndrome; toxic epidermal necrolysis; Aged; Aged, 80 and over; Antigens, Differentiation, T-Lymphocyte; Biological Markers; Biopsy, Needle; Cohort Studies; Diagnosis, Differential; Drug Eruptions; Female; Forkhead Transcription Factors; Humans; Immunohistochemistry; Male; Middle Aged; Perforin; Prognosis; Retrospective Studies; Severity of Illness Index; Skin Diseases, Vesiculobullous; Stevens-Johnson Syndrome; Young AdultGeneralized bullous fixed drug eruption is distinct from Stevens-Johnson syndrome/toxic epidermal necrolysis by immunohistopathological featuresjournal article10.1016/j.jaad.2013.11.015243887222-s2.0-84894057183