Bullous and cicatricial pemphigoid. Clinical, histopathologic, and immunopathologic correlations

A study of the records, biopsies, and immunopathologic data from 84 patients with bullous pemphigoid and 60 patients with cicatricial pemphigoid revealed that patients with bullous pemphigoid and negative or perhaps very low titer of circulating antibasement-membrane antibodies may constitute a distinct subgroup of patients with bullous pemphigoid. This group contained more women than men, had increased mucous membrane involvement, and had higher incidences of hypothyroidism, positive antinuclear antibody tests, and malignancy than did the other patients with bullous pemphigoid. Six of 41 patients with bullous pemphigoid who were given sulfapyridine or dapsone responded to these drugs, and these 6 constitute another group of patients necessitating further study. In nine patients, localized pemphigoid was found. The lesions were of two types: those occurring in sun-exposed areas and those occurring pretibially. Three of 57 patients with cicatricial pemphigoid had circulating antibasement-membrane antibodies. The clinical extent of disease in cicatricial pemphigoid was less extensive in our series than that previously seen at our institution. This was probably due to the earlier and more accurate diagnosis afforded by immunopathologic studies and to wider appreciation of the disease among nondermatologists.