Sir,

Wegener's granulomatosis (WG) is a multisystemic disease that affects small vessels. The necrotizing granulomatous inflammation commonly involves the upper airways, lung, and kidney, although any organ can be affected.1 Xanthogranuloma formation is a rare manifestation of the disease.1

Ocular manifestations occurs in approximately 30–50% of cases.2 The lacrimal drainage system is usually affected by direct spread of the inflammatory process from the upper airways.3 Focal vasculitis of the lacrimal sac wall is extremely rare in WG. Ghanem et al,3 described the first case in 2004.

Case report

A 21-year-old man presented with acute right dacryocystitis. He was successfully treated with intravenous antibiotics for a week, and then oral antibiotics for another 2 weeks. Incision and drainage of the localized abscess was also performed. However, the patient persisted with epiphora in the affected side. A firm, nontender swelling of the right lacrimal sac could be palpated. Lacrimal probing and irrigation demonstrated right lower lacrimal obstruction. The patient was being treated for WG with methotrexate and oral prednisolone since the previous year.

Dacryocystorhinostomy has been an effective therapy for lacrimal duct obstruction in patients with WG.4 In this patient, endonasal dacryocystorhinostomy was performed. During the procedure, a yellowish mass could be seen at the lacrimal sac wall (Figure 1). The lesion was excised and sent for histopathological evaluation. The rest of the surgery was uneventful. In addition to the small cell vasculitis (Figure 2), a proliferation of xanthomized histiocytes (Figures 3 and 4) could be seen. Touton or Langerhans multinucleated giant cells were not observed. Special stains for microorganisms failed to reveal any infectious aetiology. The histiocytes did not stain for PAS, thus excluding Whipple's disease. Given the history of WG and no other finding that could suggest a different cause for the xanthogranuloma, the relationship was established. At follow-up, the lacrimal drainage system is patent 8 months after the procedure.

Figure 1
figure 1

Endonasal dacryocystorhinostomy. A yellowish mass is seen in the lacrimal sac wall (white arrow). Nasal septum (white asterisk) and middle turbinate (black arrow).

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Figure 2
figure 2

Photomicrography. Chronic inflammatory infiltrate destroying the wall of a small vessel of the lacrimal sac (H&E, original magnification, × 400).

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Figure 3
figure 3

Photomicrography. A well-delimited aggregate of xanthomized histiocytes (arrowheads) is seen at the lacrimal sac wall. Immunohistochemistry was positive for CD68 (H&E, original magnification, × 200).

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Figure 4
figure 4

Photomicrography. Higher magnification of the same area showed in Figure 3. The xanthomized histiocytes appear pale with small nuclei and vacuolated cytoplasm (H&E, original magnification, × 400).

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Comment

The xanthomatous transformation in WG is considered to be a focal accumulation of lipid-rich histiocytes secondary to the necrotizing vasculitis, and corresponds to the xanthoma of the skin, described by Frances et al.1 To the best of our knowledge, a xanthogranuloma of the lacrimal sac as a manifestation of WG has never been described before.