Atypical clinical appearance of pemphigus vulgaris on the face: case report

Pemphigus vulgaris is an organ-specific autoimmune mucocutaneous disorder. In the majority of cases, the disease manifests initially with oral lesions, and may be limited to a single site for months before spreading. A 78-year-old woman with yellowish crusted areas on her left preauricular region and close to the medial angle of her right eye is presented. Although she described an episode of erosions on her lower lip, the involvement of mucosal surfaces was not noticed on examination. Before she presented to our Department, she was misdiagnosed as an actinic cheilitis and malignant skin tumor. Histopathologic examination and direct immunofluorescence confirmed the diagnosis of pemphigus vulgaris. Immunoblotting of epidermal extracts detected IgG antibodies against desmoglein 3 but not desmoglein 1, which was also confirmed by ELISA test. The patient responded favorably to systemic corticosteroid therapy combined with adjuvant immunosuppressive therapy, with complete clearance of the lesions.