Pemphigus mimicking common skin diseases--atypical presentation delaying correct diagnosis: case series of five patients.

摘要

Pemphigus, a rare skin disorder, encompasses a class of mucocutaneous autoimmune blistering diseases characterized by loss of cell-cell adhesion (acantholysis) mediated by autoantibodies to epidermal cell surface proteins [1]. The presence of flaccid non-inflammatory blisters and erosions that arise on normal-appearing skin and variable involvement of the mucous membranes characterize its three major variants: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. The diagnosis of pemphigus is based on three key elements: a) the presence of typical clinical lesions, b) demonstration of acantholysis on skin biopsy, and c) the finding of anti-desmoglein autoantibodies in tissue, whether present in the patients' serum or not.