title: Clinical and immunological profiles in 17 Japanese patients with drug-induced pemphigus studied at Kurume University.
abstract: BACKGROUND
  Drug-induced pemphigus (DIP) shows clinical, histopathological and immunological features of pemphigus. However, little is known about immunological profiles in DIP.
  
  
  OBJECTIVE
  To characterize clinical and immunological profiles in patients with DIP.
  
  
  METHODS
  We studied 17 Japanese patients with DIP who were treated at Kurume University Hospital or who consulted from other hospitals between 1997 and 2012. Complicated diseases, clinical and histopathological manifestations, responsible drugs and findings in immunofluorescence, enzyme-linked immunosorbent assays (ELISAs), immunoblotting (IB) and prognosis were analysed.
  
  
  RESULTS
  Eight of the 17 patients with DIP showed pemphigus foliaceus-like appearance, three showed pemphigus herpetiformis-like appearance, and six showed atypical bullous lesions. Responsible drugs were thiol-containing drugs in 16 patients (bucillamine in nine cases, d-penicillamine in four cases, and cetapril, thiopronine and captopril in one patient each), and a nonthiol drug, sulfasalazine, in one patient. By ELISAs and/or IB analyses, nine patients reacted only with desmoglein 1 (Dsg1), four reacted with Dsg1 and Dsg3, and four showed no specific reactivity. By IB of normal human epidermal extracts, in addition to positive reactivity with Dsg1, four patients with no detectable malignancy showed paraneoplastic pemphigus-like reactivity with the 210-kDa envoplakin and the 190-kDa periplakin. Four cases showed anti-Dsg3 antibodies without mucosal lesions. While 11 cases recovered after discontinuation of the causative drugs, six patients had a very protracted or intractable disease course, and might develop true pemphigus.
  
  
  CONCLUSIONS
  The present study indicated that the majority of the patients with DIP studied showed a pemphigus foliaceus-type phenotype with anti-Dsg1 autoantibodies, caused by thiol-containing drugs.
authors.affiliation: Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, 67 Asahimachi, Kurume, Fukuoka, 830-0011, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Yoshimura
authors.forename: N
authors.initials: N
authors.lastname: Ishii
authors.forename: T
authors.initials: T
authors.lastname: Hamada
authors.forename: T
authors.initials: T
authors.lastname: Abe
authors.forename: F
authors.initials: F
authors.lastname: Ono
authors.forename: K
authors.initials: K
authors.lastname: Hashikawa
authors.forename: S
authors.initials: S
authors.lastname: Fukuda
authors.forename: B
authors.initials: B
authors.lastname: Ohyama
authors.forename: H
authors.initials: H
authors.lastname: Koga
authors.forename: R
authors.initials: R
authors.lastname: Sogame
authors.forename: K
authors.initials: K
authors.lastname: Teye
authors.forename: T
authors.initials: T
authors.lastname: Ochiai
authors.forename: H
authors.initials: H
authors.lastname: Nakajima
authors.forename: K
authors.initials: K
authors.lastname: Nakajima
authors.forename: S
authors.initials: S
authors.lastname: Iijima
authors.forename: M
authors.initials: M
authors.lastname: Kanzaki
authors.forename: K
authors.initials: K
authors.lastname: Kojima
authors.forename: T
authors.initials: T
authors.lastname: Nagatani
authors.forename: W
authors.initials: W
authors.lastname: Fujimoto
authors.forename: T
authors.initials: T
authors.lastname: Karashima
authors.forename: T
authors.initials: T
authors.lastname: Nakama
authors.forename: C
authors.initials: C
authors.lastname: Ohata
authors.forename: M
authors.initials: M
authors.lastname: Furumura
authors.forename: D
authors.initials: D
authors.lastname: Tsuruta
authors.forename: T
authors.initials: T
authors.lastname: Hashimoto
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000276:immunology; D051183:Desmoglein 1 / Q000276:immunology; D003875:Drug Eruptions / Q000209:etiology* / Q000378:metabolism; D005260:Female; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000139:chemically induced* / Q000276:immunology
chemical_list: D001323:Autoantibodies; D051183:Desmoglein 1