Mucous membrane pemphigoid: our 14 years`experience

Resumen

Abstract

Introduction. Mucous membrane pemphigoid includes a group of subepithelial immunobullous diseases mainly aff ecting mucous membranes and occasionally the skin. Evolution is chronic and progressive, leading to cicatricial sequelae, which produce morbidity, disability, and sometimes death.

Objective: To describe epidemiological and clinical aspects, and the evolution of mucous and cutaneous lesions in our patients.

Materials and methods. The retrospective, observational, and descriptive study included 41 patients with confi rmed mucous membrane pemphigoid diagnosis consulting the Dermatology Department between 1994 and 2008. Ocular involvement was graded using the Foster staging system. Each patient received systemic and local treatment.

Results. Thirty nine patients had only ocular involvement and 2 patients had ocular, oral and cutaneous disease, with female predominance of the disease. Mean age was 60 years, 46.34 percent had history of severe ocular injury, 17.07 percent had used topical glaucoma medications, and 80.48 percent consulted for chronic conjunctivitis. In 60.96 percent of the cases, diagnosis was confi rmed in advanced stages of ocular disease. 62.41 percent of patients had over 5 years evolution before diagnosis. Cutaneous and oral lesions responded to therapy in less than 2 months. Ocular lesions remission according to stage was as follows: stage I, 83.33 percent; stage II, 40 percent; and stage III, 30.43 percent. No immunosuppressive treatment was given in stage IV. No improvement was achieved in terminal ocular disease patients.

Conclusion. Ocular involvement is prognostic. Diagnosis was made in advanced stages of ocular disease. Remission was achieved only with early treatment.

(Dermatol Argent 2009; 15(4):260-266).

Key words: Mucous membrane pemphigoid, cicatricial pemphigoid..