Ulcerative granuloma of the eyelid as the initial manifestation of granulomatosis with polyangiitis (Wegener's granulomatosis): A case report.

A 56-year-old-man presented a 2-month history of chalazion in the eyelids without response to treatment and with an inconclusive biopsy. Laboratory results confirmed the presence of Enterobacter cloacae and Streptococcus gordonii infection. Despite appropriate intravenous antibiotic treatment, clinical worsening was observed. Radical surgical excision and total tarsorrhaphy were performed. Following treatment, the patient was asymptomatic for 6 weeks until he developed acute renal failure, generalised arthralgia, acute hypertensive anterior uveitis, and dacryoadenitis. Lacrimal gland biopsy revealed a perivascular granulomatous inflammation with necrotic foci. Renal biopsy showed a necrotizing extracapillary glomerulonephritis. Blood tests showed elevated levels of C-reactive protein, positive rheumatoid factor, and proteinase 3-anti-neutrophil cytoplasmic antibody. The patients was therefore diagnosed with granulomatosis with polyangiitis and treated with cyclophosphamide and corticosteroids, with good systemic and orbital response. Surgical reconstruction of the eyelid was subsequently performed. This case describes, for the first time in the literature, an eyelid granuloma as the presenting sign in GPA, highlighting the importance of differential diagnosis of eyelid lesions with unusual characteristics.