Cutaneous type pemphigus vulgaris

A rare clinical phenotype of pemphigus

Kazue Yoshida, Yujiro Takae, Hitoshi Saito, Hiroshi Oka, Akiko Tanikawa, Masayuki Amagai, Takeji Nishikawa

Research output: Contribution to journalArticle

51 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)839-845
Number of pages7
JournalJournal of the American Academy of Dermatology
Volume52
Issue number5
DOIs
Publication statusPublished - 2005 May

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Pemphigus
Phenotype
Skin
Desmoglein 1
Autoantibodies
Desmogleins
Immunoglobulin G
Acantholysis
Blister
Autoimmune Diseases
Mucous Membrane
Enzyme-Linked Immunosorbent Assay

ASJC Scopus subject areas

  • Dermatology

Cite this

Cutaneous type pemphigus vulgaris : A rare clinical phenotype of pemphigus. / Yoshida, Kazue; Takae, Yujiro; Saito, Hitoshi; Oka, Hiroshi; Tanikawa, Akiko; Amagai, Masayuki; Nishikawa, Takeji.

In: Journal of the American Academy of Dermatology, Vol. 52, No. 5, 05.2005, p. 839-845.

Research output: Contribution to journalArticle

Yoshida, Kazue ; Takae, Yujiro ; Saito, Hitoshi ; Oka, Hiroshi ; Tanikawa, Akiko ; Amagai, Masayuki ; Nishikawa, Takeji. / Cutaneous type pemphigus vulgaris : A rare clinical phenotype of pemphigus. In: Journal of the American Academy of Dermatology. 2005 ; Vol. 52, No. 5. pp. 839-845.
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