Cutaneous type pemphigus vulgaris: A rare clinical phenotype of pemphigus - 21/08/11
Tokyo, Japan
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.
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Supported by Health and Labour Sciences research grants for research on measures for intractable diseases from the Ministry of Health, Labor, and Welfare and by grants-in-aid for scientific research from the Ministry of Education, Culture, Sports, Science, and Technology of Japan. Conflicts of interest: None. |
Vol 52 - N° 5
P. 839-845 - mai 2005 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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