Mucous membrane pemphigoid: clinical aspects, immunopathological features and therapy

More than 50 years have passed since Civatte, Lever, and others described the clinical and histopathological characteristics of mucous membrane pemphigoid (synonym: cicatricial pemphigoid). This enigmatic, relapsing, and often eventually progressive subepithelial blistering disorder of the mucous membranes and skin continues to challenge investigators trying to understand the pathogenesis of the disease and prevent its progression. Mucous membrane pemphigoid typically begins in late adulthood and has a variable prognosis. Fifty percent of patients will develop esophageal and ocular lesions that heal with secondary atrophy leading to stenosis of the upper aerodigestive tract and blindness in uncontrolled disease. Recent progress has been made in understanding the cause, the immunological components, and the pathologic process of mucous membrane pemphigoid. The short-term clinical and pathological manifestations of disease activity have been reduced by new therapies, although the degree of long-term benefit from these treatments awaits further study.