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Oral pemphigus vulgaris. A report of ten cases.

The conditions of ten patients were diagnosed as pemphigus vulgaris limited to the oral cavity. In eight patients the histopathologic findings were consistent with oral pemphigus. Direct immunofluorescence demonstrated intercellular substance (ICS) deposition of C3 either alone or in combination with IgG, IgA, IgM, C4, C1q, properdin, and factor B in all nine patients tested. Circulating ICS antibodies were detected initially in eight of the ten patients, nine of whom had positive titers at a later date, without subsequent development of cutaneous lesions. Physicians should be alerted to a diagnosis of pemphigus in patients who have chronic erosive disease confined to the oral cavity. Prompt diagnosis by means of routine histopathologic and immunofluorescence studies followed by early initiation of treatment are warranted.