title: IgA pemphigus.
abstract: Pemphigus is a life-threatening autoimmune blistering disease. Pemphigus is divided into 4 major types; pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, and IgA pemphigus. Among them, IgA pemphigus is characterized by tissue-bound and circulating IgA antibodies targeting desmosomal or nondesmosomal cell surface components in the epidermis. Histopathologically, slight epidermal acantholysis and extensive neutrophilic infiltration in either the upper part or all layers of the epidermis were observed. IgA pemphigus is subdivided into intraepidermal neutrophilic IgA dermatosis-type (IEN-type), whose target antigen is still unknown (probably nondesmosomal cell surface protein), and subcorneal pustular dermatosis-type (SPD-type), whose target antigen is desmocollin 1 (Dsc1). We summarize reported cases of IgA pemphigus and describe current knowledge including epidemiology, clinical manifestations, pathology, laboratory tests, pathophysiology, associated diseases, prognosis and treatment, and future perspectives of IgA pemphigus.
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: Daisuke
authors.initials: D
authors.lastname: Tsuruta
authors.forename: Norito
authors.initials: N
authors.lastname: Ishii
authors.forename: Takahiro
authors.initials: T
authors.lastname: Hamada
authors.forename: Bungo
authors.initials: B
authors.lastname: Ohyama
authors.forename: Shunpei
authors.initials: S
authors.lastname: Fukuda
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Koga
authors.forename: Kazuko
authors.initials: K
authors.lastname: Imamura
authors.forename: Hiromi
authors.initials: H
authors.lastname: Kobayashi
authors.forename: Tadashi
authors.initials: T
authors.lastname: Karashima
authors.forename: Takekuni
authors.initials: T
authors.lastname: Nakama
authors.forename: Teruki
authors.initials: T
authors.lastname: Dainichi
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D051187:Desmocollins / Q000276:immunology; D003896:Desmosomes / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology*; D015994:Incidence; D007223:Infant; D008297:Male; D008875:Middle Aged; D009504:Neutrophils / Q000276:immunology / Q000473:pathology; D010392:Pemphigus / Q000145:classification / Q000453:epidemiology / Q000276:immunology / Q000473:pathology; D015995:Prevalence; D055815:Young Adult
chemical_list: D001323:Autoantibodies; C081560:DSC1 protein, human; D051187:Desmocollins; D007070:Immunoglobulin A