[Cicatricial pemphigoid--an important differential diagnosis in inflammatory mucous membrane changes]

Cicatricial pemphigoid is a chronic autoimmune subepithelial bullous disease that primarily involves mucous membranes and less frequently the skin. The subepithelial blisters characteristically heal with scarring.

In seven years, eleven patients (ten female, one male) underwent interdisciplinary treatment in the departments of dermatology, otorhinolaryngology, and ophthalmology of the university of Würzburg. The mean age of onset was 69 (50-76) years. Histopathologic examination and direct immunofluorescence microscopy of skin biopsies and indirect immunofluorescence analysis of sera were performed in all patients. Seven patients were treated with dexamethasone and cyclophosphamide, two patients received dapsone and two patients both therapy schemes.

The oral cavity (n = 10) and/or conjunctival mucosa (n = 6) was most frequently involved, less frequently the larynx (n = 4), the nasal mucosa (n = 3), the skin (n = 3), or the pharynx (n = 1). Histopathologic examination of lesional skin biopsies revealed subepithelial blisters accompanied by mixed mononuclear inflammatory cell infiltrates. Direct immunofluorescence microscopy of skin adjacent to the lesion showed linear deposits of IgG and/or C3 along the cutaneous basement membrane zone. Circulating anti-basement membrane zone autoantibodies were detected in six patients, binding on the dermal (n = 3), epidermal (n = 2), or epidermal/dermal (n = 1) side of NaCl-separated human split skin. Though mucosal and skin lesions responded well to treatment, the ocular involvement was frequently progressive under the therapy, even with a combined dexamethasone and cyclophosphamide pulse regimen.

The various clinical symptoms may make the diagnosis of cicatricial pemphigoid difficult. A detailed history and physical examination and application of direct and indirect immunofluorescence microscopy are important for the accurate diagnosis. A spontaneous remission of cicatricial pemphigoid is uncommon. Because of treatment limitations in the later stages of the disease, adequate therapy should start early.