Purpose: To report the occurrence of ocular involvement in the setting of pemphigus and discuss its relationship with disease activity and prognostic significance.
Design: Retrospective case reports.
Methods: Five patients, aged 38 to 65 years, diagnosed with pemphigus according to clinical, histopathologic, and immunopathologic criteria (n = 4 pemphigus vulgaris; n = 1 superficial pemphigus) developed ocular symptoms and signs consistent with the disease, ranging from mild conjunctivitis to blisters and prominent erosions of the bulbar/palpebral conjunctiva or at the eyelid margin.
Results: Ocular involvement in our series mostly followed skin disease or represented the stigmata of quiescent localized pemphigus. One of five patients had fatal outcome from myocardial infarction, whereas in the remaining cases significant improvement was achieved with oral prednisolone.
Conclusions: Ocular pemphigus is probably underdiagnosed and its frequency appears underestimated. It does not seem to correlate with disease severity, but may persist chronically after healing of cutaneous lesion.