Pemphigus vegetans

Orpha code: 79479OMIM code:

Definition

A rare autoimmune bullous skin disease characterized by mucocutaneous bullae with subsequent erosion and formation of vegetative plaques, predominantly affecting intertriginous areas and the oral mucosa. Two clinical forms of the disease are recognized: the Hallopeau type, which presents an indolent course with pustules healing as vegetative plaques and frequent lack of involvement of the oral mucosa, and the Neumann type, which takes a more severe, refractory course with vegetations developing during an eruption of vesiculobullous lesions and involvement of the oral mucosa. Serum analysis reveals antibodies against desmoglein 1 and 3.

Disease data
Classification

Disease

ORPHA code
79479
OMIM code
-
ICD10 code
L10.1
ICD11 code
EB40.0Y

No additional description.

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