Mucous membrane pemphigoid

Abstract

Autoantibodies to one or several autoantigens in the mucosal or epithelial BMZ have been identified in MMP patients. 4-10 The association of MMP with human leukocyte antigen (HLA) major histocompatibility class II HLA-DQB1*0301 has been demonstrated. 11-13 The cause is usually unknown, but there are a few reports of MMP triggered by medications such as methyldopa, clonidine and D-penicillamine. 14, 15 EPIDEMIOLOGY The true incidence of MMP is unclear. A recent study from the United Kingdom demonstrated that ocular MMP accounted for 61% of the cases of newly diagnosed cicatricial conjunctivitis and the incidence was calculated as 0.8 per million population. 16 The incidence of MMP was estimated to be 1.3-2.0 per million per year in France and Germany. 17, 18 MMP predominantly affects women more often than men with a male to female ratio of nearly 2:1. 19 MMP mainly occurs in the elderly population, commonly observed between 60 and 80 years of age. 20 Albeit rare, children may also be affected. Approximately 20 cases of childhood onset MMP have been reported, among whom the youngest one was 10 months old. 21-23 There is no known racial or geographic predilection.