dermatologic disorders
Mucous Membrane Pemphigoid
Introduction
Mucous membrane pemphigoid is the designation given to a heterogeneous group of rare chronic autoimmune disorders that tend to cause waxing and waning bullous lesions of the mucous membranes, often with subsequent scarring and morbidity. Diagnosis is by skin biopsy and direct immunofluorescence. Topical or intralesional corticosteroids and a combination ofdoxycyclineand nicotinamide may be used for mild disease. Systemic immunosuppression may be needed for severe disease. Bullae are elevated, fluid-filled blisters ≥ 10 mm in diameter. (Synonyms of mucous membrane pemphigoid that are no longer used include cicatricial pemphigoid, ocular cicatricial pemphigoid, and benign mucous membrane pemphigoid.) Oral mucous membrane pemphigoid and Ocular Mucous Membrane Pemphigoid are typical, but other mucosal sites and the skin (usually of the head and upper trunk) may be involved. Older people are most often affected, women more than men. Mucous membrane pemphigoid (MMP) is characterized by subepithelial lesions caused by autoantibodies against molecules of epithelial basement membranes. The target molecules lie deeper than those of Bullous Pemphigoid . Several autoantibodies have been identified, including those against BPAG2, laminin-332, and type VII collagen. Antibodies to beta-4 integrin have been identified in generalized MMP and ocular MMP, and antibodies to alpha-6 integrin have been identified in oral MMP. 