title: Cutaneous type pemphigus vulgaris: a rare clinical phenotype of pemphigus.
abstract: Pemphigus is an autoimmune blistering disease of the skin, mucous membranes, or both. There are two main categories of pemphigus: pemphigus foliaceus (PF) and pemphigus vulgaris (PV). PV is further subdivided into mucosal dominant and mucocutaneous types, according to the extent of cutaneous lesions. These classes of pemphigus have distinct histopathologic and serologic findings, with most cases falling into these subtypes. We report 4 cases that clinically showed blisters and erosions in the skin only, without mucosal involvement. Histologic examination of cutaneous lesions demonstrated suprabasilar acantholysis, a typical finding for PV. These patients had predominant anti-desmoglein 1 (Dsg1) IgG autoantibodies as well as anti-Dsg3 IgG autoantibodies, as determined by enzyme-linked immunosorbent assay. The desmoglein compensation theory posits that this rare phenotype can be produced by pathogenically weak anti-Dsg3 IgG in the presence of potent anti-Dsg1 IgG autoantibodies. Thus, cutaneous type PV without apparent mucosal involvement is observed as a rare clinical and histologic expression of pemphigus. This expression can be a transient phenotype that may develop from, or evolve into, other subtypes of pemphigus.
authors.affiliation: Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Kazue
authors.initials: K
authors.lastname: Yoshida
authors.forename: Yujiro
authors.initials: Y
authors.lastname: Takae
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Saito
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Oka
authors.forename: Akiko
authors.initials: A
authors.lastname: Tanikawa
authors.forename: Masayuki
authors.initials: M
authors.lastname: Amagai
authors.forename: Takeji
authors.initials: T
authors.lastname: Nishikawa
mesh_terms: D001323:Autoantibodies / Q000032:analysis; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000276:immunology* / Q000473:pathology
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G