Pemphigus vulgaire de présentation inhabituelle

Abstract

Pemphigus vulgaris is a bullous auto-immune disease affecting the skin and mucosa. It is characterised by acantholysis that results in the formation of intraepithelial bullous lesions. Herein we report a case distinguished by its unusual clinical presentation. A 45-year-old man, a chronic smoker, consulted for hyperkeratotic lesions of the toes on the right foot present for 5months. Examination revealed a violet colour of the toes associated with localised yellowish keratoderma on the sole of the foot and impaired toe nails, as well as impairment on the nails of the first, third and fourth fingers on the right hand. In addition, two hyperkeratotic plaques with crusts were noted on the patient's forehead. Histological examination of a biopsy sample taken from the nail bed of the fourth toe on the right foot showed suprabasal acantholysis with a characteristic tombstone appearance. Direct immunofluorescence confirmed the diagnosis of pemphigus. Oral corticosteroid therapy was initiated consisting of prednisone 1.5mg/kg per day. Improvement of the nail lesions and subsidence of the lesions on the patient's forehead occurred after three months of treatment. During the course of pemphigus, the nail findings most commonly reported in the literature are paronychia, onychomadesis and onycholysis. However, these signs are generally seen in patients with known pemphigus vulgaris and only rarely indicate bullous disease. The case we report illustrates a special situation in which pemphigus vulgaris was revealed by unusual skin and nail lesions.