Clinical, historic, and therapeutic features of cicatricial pemphigoid. A literature review and open therapeutic trial with corticosteroids.

Cicatricial pemphigoid is at present an incurable, autoimmune disease that involves mucosa and skin. We have documented the clinical, microscopic, and therapeutic features of 23 patients with cicatricial pemphigoid and added these to reports of past literature. The mean age at the time of diagnosis was 63 years, and women were involved twice as often as men. Eighty-three percent of patients had oral mucosal involvement, 70% had conjunctival involvement, and 22% had skin involved. Direct immunologic evaluation revealed IgG at the basement membrane in 57% of cases and C3 in 66%. We have been able to manage the disease adequately in most patients with topical or systemic corticosteroids. The most commonly encountered side effect was oral candidiasis.