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Case Reports

Abstract

Pemphigus vulgaris (PV) is a rare autoimmune blistering disorder characterized by suprabasilar acantholysis and circulating antibodies against desmogleins. While its clinical and histopathologic features are generally well defined, atypical presentations can pose significant diagnostic challenges. We report a unique case of PV presenting with segmental distribution and histologic overlap with both Darier’s disease and Hailey-Hailey disease.

A 31-year-old female initially presented with scattered hyperkeratotic papules arising within a tattoo. A biopsy from a lesion on the right jaw demonstrated papillomatosis with suprabasilar acantholysis and dyskeratosis, associated with overlying crust and secondary impetiginization. The differential diagnosis at that time included an atypical epidermal nevus, segmental Darier’s disease, and, less likely, an immunobullous disorder.

The patient subsequently developed additional lesions on the upper back characterized by crusted superficial erosions in a seborrheic distribution, and blisters involving the hands and oral mucosa. A second biopsy revealed suprabasilar acantholysis with acantholytic dyskeratosis, findings consistent with a variant of pemphigus.

Direct immunofluorescence was positive, and serologic studies confirmed elevated anti-desmoglein 1 antibody. While the histopathology supported a diagnosis of pemphigus, overlapping features with Hailey-Hailey and Darier’s disease were also noted—an unusual finding that raises the possibility of either a rare pemphigus variant or a concurrent genodermatosis. 

The patient was referred for consideration of rituximab therapy. She is currently experiencing an active flare involving the back, scalp, and face, despite ongoing mycophenolate mofetil (CellCept) treatment at 1500 mg daily.

This case highlights the importance of distinguishing autoimmune blistering disorders from inherited acantholytic dermatoses.