title: Generalized bullous fixed drug eruption is distinct from Stevens-Johnson syndrome/toxic epidermal necrolysis by immunohistopathological features.
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.
  
  
  CONCLUSIONS
  The number of cases in this study is small.
  
  
  CONCLUSIONS
  GBFDE is a distinct disease distinguishable from SJS/TEN by particular features such as granulysin, CD56, and Foxp3 expressions.
authors.affiliation: Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
authors.forename: Yung-Tsu
authors.initials: YT
authors.lastname: Cho
authors.affiliation: 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.
authors.forename: Jheng-Wei
authors.initials: JW
authors.lastname: Lin
authors.affiliation: Department of Dermatology, Cathay General Hospital, Taipei, Taiwan.
authors.forename: Yi-Chun
authors.initials: YC
authors.lastname: Chen
authors.affiliation: Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
authors.forename: Chia-Ying
authors.initials: CY
authors.lastname: Chang
authors.affiliation: Department of Pathology, Cheng Shin General Hospital, Taipei, Taiwan.
authors.forename: Cheng-Hsiang
authors.initials: CH
authors.lastname: Hsiao
authors.affiliation: 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.
authors.forename: Wen-Hung
authors.initials: WH
authors.lastname: Chung
authors.affiliation: Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan. Electronic address: chiayu@ntu.edu.tw.
authors.forename: Chia-Yu
authors.initials: CY
authors.lastname: Chu
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000945:Antigens, Differentiation, T-Lymphocyte / Q000235:genetics / Q000378:metabolism*; D015415:Biomarkers / Q000097:blood; D001707:Biopsy, Needle; D015331:Cohort Studies; D003937:Diagnosis, Differential; D003875:Drug Eruptions / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D051858:Forkhead Transcription Factors / Q000032:analysis / Q000097:blood; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D054353:Perforin / Q000032:analysis / Q000097:blood; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D012872:Skin Diseases, Vesiculobullous / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D013262:Stevens-Johnson Syndrome / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D055815:Young Adult
chemical_list: D000945:Antigens, Differentiation, T-Lymphocyte; D015415:Biomarkers; C418974:FOXP3 protein, human; D051858:Forkhead Transcription Factors; C075005:GNLY protein, human; D054353:Perforin
keywords: Stevens-Johnson syndrome; fixed drug eruption; generalized bullous fixed drug eruption; granulysin; regulatory T cells; toxic epidermal necrolysis