Enigma of ocular cicatricial pemphigoid. A comparative study of clinical and immunological findings.

The clinical and immunological findings in 22 cases of ocular cicatricial pemphigoid are presented. Males and females were equally affected. Mucosal ulceration (both conjunctival and oral) and early involvement of the canthal structures were the most helpful clinical signs. None of the patients had circulating antibody to basement membrane determinants at presentation, but four developed typical pemphigoid antibodies during observation and three others showed an unusual "string of pearls" pattern on indirect immunofluorescence which changed to a pemphigoid pattern in one of them. Treatment of the acute ulcerative lesions with topical steroids was effective and prevented scarring, but otherwise treatment was ineffectual. The presence of antibody and its titre was not influenced by the severity of the disease or by systemic treatment.