Update on mucous membrane pemphigoid: a heterogeneous immune-mediated subepithelial blistering entity

Abstract

Most oral involvement in the skin diseases (dermatoses) is related to mucous membrane pemphigoid or lichen planus. Mucous membrane pemphigoid was the subject of a European Symposium held in Turin, Italy, in June 1997. This review is based on that symposium. Mucous membrane pemphigoid is a subepithelial vesiculobullous disorder mainly of late middle age; it has a slight predilection for women. Whereas mucous membrane pemphigoid was formerly considered a single entity, it is now quite evident that a number of subepithelial vesiculobullous disorders may produce similar clinical pictures and also that a range of variants of mucous membrane pemphigoid exists, with antibodies directed against various hemidesmosomal components or components of the epithelial basement membrane. The term immune-mediated subepithelial blistering diseases has therefore been used. Diagnosis and management of immune-mediated subepithelial blistering diseases on clinical grounds alone are impossible; a full history, general and oral examinations, and biopsy with immunostaining are now invariably required, sometimes supplemented with other investigations. Most patients with mucous membrane pemphigoid affecting the mouth manifest desquamative gingivitis, a fairly common complaint typically seen in women who are middle-aged or older. Oral vesicles and erosions may also occur, and there can be a positive Nikolsky sign. Some patients have lesions of other stratified squamous epithelia, presenting as conjunctival, nasal, oesophageal, laryngeal, vulval, penile, or anal involvement. Apart from improving oral hygiene, immunomodulatory-in particular, immunosuppressive-therapy is typically required to control oral lesions in mucous membrane pemphigoid. No single treatment regimen reliably controls all these disorders.