An atypical case of cicatricial pemphigoid vegetans in a young male and a review of the literature

Abstract

Mucous membrane pemphigoid (MMP), previously known as cicatricial pemphigoid, is the second most frequent autoimmune subepithelial blistering disorder after bullous pemphigoid (BP). MMP has a broad spectrum of both clinical and immunological features, mostly affecting the mucous membranes, mainly the oral and ocular mucosae, but around 30% of cases develop skin lesions, which heal leaving atrophic scars and milia. Herein, we report a patient with widespread vegetating cutaneous and oral plaques, oral erosions, scarring conjunctivitis, and extensive mucosal lesions, who met the immunologic and microscopic criteria of cicatricial pemphigoid vegetans (CPV). The vegetative form of MMP is a very rare entity and, to our knowledge, this is the fifth patient recorded in the global literature. We present the unusual case of a young male who developed CPV possibly caused by chronic and heavy cannabis use and who was treated effectively with rituximab.