title: A unique case of intra-epidermal neutrophilic dermatosis-type IgA pemphigus presenting with subcorneal pustules.
abstract: BACKGROUND
  While subcorneal pustular dermatosis-type IgA pemphigus shows subcorneal pustules, intra-epidermal neutrophilic dermatosis (IEN)-type IgA pemphigus is characterised by pustule formation throughout the entire epidermis.
  
  
  OBJECTIVE
  We analysed an unusual case of IEN-type IgA pemphigus showing subcorneal pustules.
  
  
  METHODS
  Indirect immunofluorescence, immunoblot analysis, auto-antibody-specific ELISAs, and double-labelling immuno-electron microscopy were performed.
  
  
  RESULTS
  IgA antibodies bound to the surface of keratinocytes distributed throughout the epidermis were detected by indirect immunofluorescence. IgA from the patient's serum failed to react with COS-7 cells expressing desmocollins (Dsc) 1, 2 or 3. IgG or IgA antibodies against desmogleins 1 and 3 were also found to be absent by ELISA. Double-labelling immuno-electron microscopy using an anti-Dsc monoclonal antibody showed localisation in the desmosomes, while IgA from the patient's serum was found in non-desmosomal regions.
  
  
  CONCLUSIONS
  The patient in the present study most likely suffers from IEN-type IgA pemphigus.
authors.affiliation: Division of Dermatology, Showa University Fujigaoka Hospital, Yokohama, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Iida
authors.forename: Hirohiko
authors.initials: H
authors.lastname: Sueki
authors.forename: Bungo
authors.initials: B
authors.lastname: Ohyama
authors.forename: Norito
authors.initials: N
authors.lastname: Ishii
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
mesh_terms: D001327:Autoimmune Diseases / Q000473:pathology*; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009504:Neutrophils; D010392:Pemphigus / Q000276:immunology* / Q000473:pathology*
chemical_list: D007070:Immunoglobulin A