Cicatricial pemphigoid (CP) is an uncommon autoimmune vesiculobullous disease that most commonly involves the mucous membranes of the mouth and eye. Treatment for CP varies, depending on location and severity; however, systemic therapy with immunosuppressive agents is usually indicated for disease that is rapidly progressing, recalcitrant to conservative treatment, or sight threatening. We report a case of ocular CP successfully treated with a combination of oral prednisone, low-dose oral cyclophosphamide, and high-dose pulse intravenous (IV) cyclophosphamide therapy.
Report of a Case.
A 66-year-old white woman was first seen in August 1992 with a 2-month history of a blistering dermatosis in her mouth. Initial physical examination findings revealed a tense blister on the right lower gingival mucosa. An oral mucosal biopsy sample showed epithelial subbasilar cleavage, consistent with CP. Topical treatment with 0.05% fluocinonide (Lidex gel, Syntex Laboratories Inc, Palo Alto, Calif) and 0.05% halobetasol propionate (Ultravate ointment, Westwood-Squibb Pharmaceuticals Inc, Buffalo, NY) was begun; however, over the next year