title: Clinical and immunological studies for 105 Japanese seropositive patients of epidermolysis bullosa acquisita examined at Kurume University.
abstract: Using our serological diagnostic criteria, we selected 105 Japanese patients with epidermolysis bullosa acquisita (EBA), an autoimmune bullous disease (AIBD) reacting with type VII collagen, from our cohort of 5063 AIBD patients.
  
  
  
  We examined the patients clinically and immunologically.
  
  
  
  We found diversity of clinical manifestations in both cutaneous and oral mucosal lesions and a high rate of inflammatory-type EBA patients in Japan. Common treatments were systemic steroids, followed by immunosuppressives, DDS, tetracycline/minocycline and colchicine. Immunological studies revealed that indirect immunofluorescence of 1M-NaCl-split skin, immunoblotting of dermal extract, and type VII collagen ELISA were sensitive methods, with possible multiplicity of circulating autoantibodies against other basement membrane autoantigens.
  
  
  
  The present study analyzed the largest cohort of EBA patients, confirming the scarcity of EBA (only 105 of the 5063 AIBD patients), and showed that the three serological tests are useful for the diagnosis of EBA.
authors.affiliation: a Department of Dermatology , Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology , Fukuoka , Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.affiliation: a Department of Dermatology , Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology , Fukuoka , Japan.
authors.forename: Zhexiong
authors.initials: Z
authors.lastname: Jin
authors.affiliation: a Department of Dermatology , Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology , Fukuoka , Japan.
authors.forename: Norito
authors.initials: N
authors.lastname: Ishii
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D015331:Cohort Studies; D024143:Collagen Type VII / Q000276:immunology*; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009061:Mouth Mucosa / Q000473:pathology*; D015995:Prevalence; D012698:Serologic Tests; D012867:Skin / Q000473:pathology*; D013256:Steroids / Q000627:therapeutic use; D013752:Tetracycline / Q000627:therapeutic use; D014495:Universities
chemical_list: D001323:Autoantibodies; D024143:Collagen Type VII; D007166:Immunosuppressive Agents; D013256:Steroids; D013752:Tetracycline
keywords: Autoantigens; ELISA; autoimmune bullous diseases; basement membrane zone; diagnosis; epidermolysis bullosa acquisita; immunoblotting; immunofluorescence; treatment; type VII collagen