Ocular pemphigus

Background: Ocular involvement in patients with pemphigus vulgaris (PV) has been rarely reported. We report ocular involvement in 11 patients with PV. Methods: Medical records of 11 biopsy-proven patients with PV treated during the period between 1990 and 2003 were reviewed and clinical information was analyzed. Results: Mean age at onset of PV was 52.3 years (range, 30-80 years). Ocular disease was preceded by involvement of the skin, other mucosae, or both in all patients. Ocular involvement was limited to the conjunctivae, the eyelids, or both. PV did not affect the visual acuity of any of the patients. Suprabasal acantholysis was observed on routine histologic examination of the conjunctiva and skin of the eyelid. Direct immunofluorescence of perilesional eyelid skin demonstrated deposition of IgG on epithelial cell surface. Mean duration of follow up was 48.9 months (range, 4-100 months). Recurrence of ocular disease occurred in 3 patients; recurrence at nonocular mucosae occurred in 4 patients. No sequelae were observed during detailed follow-up. Ocular pemphigus improved with systemic therapy. The mean remission period was 32 months (range, 0-92 months). Conclusions: Ocular involvement in PV is rare. Involvement is limited to the conjunctiva, the eyelids, or both. PV does not appear to affect visual acuity. Patients have full recovery without sequelae.