Sir,
A 79-year-old gentleman presented with a left upper lid lump clinically typical of a chalazion. Incision and curettage was performed twice, and each time followed by recurrence. The second time, the lid lesion had grown to a very large size and filled 3/4 of the upper lid and produced a considerable ptosis (Figure 1a). The lesion was incised via a skin incision and curettings were sent for histology together with a tarsal plate biopsy. The histology showed extensive tissue necrosis associated with a heavy eosinophil infiltrate (Figure 1b). Neither active vasculitis nor granulomatous inflammation was identified, but the appearances were considered to reflect tissue involvement in Wegener's granulomatosis (WG), particularly in view of similar findings in a previous prostatic biopsy. Relevantly, the past medical history included previously active WG, which presented as prostatic enlargement. Circulating anti-neutrophil cytoplasmic antibody (cANCA) was high, but there was no other evidence of clinically active WG elsewhere in the body. The patient was commenced on 30 mg prednisolone and azathioprine. With continuing systemic immunosuppression, over the next 4 months, complete resolution of the lesion followed (Figure 1c).
Comment
Chalazion is the most common inflammatory lesion of the eyelid,1 but can occasionally be clinically confused with other benign or malignant lesions—sebaceous gland carcinoma being the most frequently misdiagnosed malignancy.2 WG is a multisystem disease of unknown aetiology characterized by granuloma formation, necrosis and variable degrees of small to medium-sized vessel vasculitis.3 Clinical presentation is most common with initial upper or lower respiratory tract, renal, or orbital symptoms.4 The main orbital signs of the disease are proptosis, scleritis, and lid inflammation,5 but WG mimicking the presentation of a chalazion has not been reported in the literature. Biopsy results in cases of WG with orbital involvement can be difficult to interpret as there are a spectrum of histopathological features, and the classic triad of vasculitis, tissue necrosis, and granulomatosis inflammation may only be present in about one-half of cases.6 A positive assay for cANCA with specificity for proteinase-3 or myeloperoxidase provides strong evidence if WG is suspected. Diagnosis of orbital WG therefore requires correlation of the clinical signs, use of the cANCA test and comparison with extraorbital biopsies, if available. This case highlights an atypical presentation of WG to the ophthalmologist, with resolution after conventional systemic treatment of the underlying disorder.
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Presented at the British Oculoplastic Surgery Society Meeting 2006
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Ismail, A., Theaker, J. & Manners, R. Wegener's granulomatosis masquerading as upper lid chalazion. Eye 21, 883–884 (2007). https://doi.org/10.1038/sj.eye.6702758
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DOI: https://doi.org/10.1038/sj.eye.6702758
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