Purpose: To describe a patient with Wegener granulomatosis (WG) previously in remission who developed conjunctival ulceration as the first sign of disease recurrence.
Methods: Case report and review of the literature.
Results: Twenty-one years after WG was originally diagnosed and with the disease thought to be in remission, a 52-year-old man with complaints of ocular irritation for the previous year was found to have multiple palpebral conjunctival ulcerations of the left eye. Incisional biopsy revealed mixed inflammation consistent with WG. Within 3 months of recognition of his conjunctival ulcers, newly recurrent pulmonary inflammation developed and serologies for cytoplasmic-pattern antineutrophil cytoplasmic antibodies (C-ANCA) became positive.
Conclusion: Conjunctival ulceration is a rare manifestation of WG but may presage more widespread disease. Mucosal ulceration in a patient with a previous diagnosis of WG should stimulate an aggressive search for renewed systemic disease activity.