Cicatricial pemphigoid

Cicatricial pemphigoid is a subepidermal blistering disease that involves the mucous membranes and the skin. The oral cavity and the eye are most frequently involved. The clinical course is of long duration, and often there is significant scarring that can have devastating sequelae. The majority of the patients are elderly. The disease is characterized by the in vivo deposition of an anti-basement membrane zone antibody. The anti-basement membrane zone antibody cannot be detected in the circulation by routine laboratory techniques. The pathogenesis is poorly understood, and the cause is not known. Cicatricial pemphigoid may remain localized to the oral cavity or the eye or the skin (Brunsting-Perry variety), or it may be generalized. It rarely occurs in children, and it may be drug induced. Efforts must be made to differentiate cicatricial pemphigoid from bullous pemphigoid, epidermolysis bullosa acquisita, linear IgA bullous disease, and other vesiculobullous disease. Early recognition and treatment can improve the prognosis and avoid surgical intervention. Topical therapy is beneficial and expedites healing. Intralesional corticosteroids are effective and can help reduce the dose of systemic steroids. Most patients require systemic corticosteroid therapy. Dapsone is also useful in treating cicatricial pemphigoid, especially in patients in whom systemic steroids are ineffective or in whom they have to be discontinued because of side effects. Immunosuppressive agents (azathioprine or cyclosphosphamide) are indicated in patients with progressive disease. Occasionally both drugs may be needed.