A 44-year-old woman presented with a 3-week history of fever, dyspnoea, rhinorrhoea, and ear and left superior eyelid pain despite antibiotic regimens. Physical examination revealed a left media otitis, left dacryoadenitis with upper eyelid perforation (Fig. 1a, b, arrow), strawberry gingivitis, and nasal crusts (c, arrows). Usual biochemical, immunologic, and infectious blood and urine tests were unremarkable, excepted increased C-reactive protein and proteinase 3(PR3)-antineutrophil cytoplasmic antibody (ANCA) levels. A thoracic CT scan showed nodular parenchymal condensation in the left lower lobe, and a bronchoscopy found diffuse bronchial infiltration of which biopsy exhibited inflammatory necrotic granulomas without identified vasculitis. The diagnosis of granulomatosis with polyangiitis (GPA) with ulcerative dacryoadenitis resulting in palpebral perforation was retained. Complete remission, including the full healing of the palpebral involvement within 4 weeks (d, arrow), was achieved after a combination of rituximab 375 mg/m2 and corticosteroids (3 boli of methylprednisolone followed by prednisone at 1 mg/kg daily, capped at 60 mg).

Fig. 1
figure 1

Left upper eyelid perforation (a, b, arrows) with full healing after treatment (d). Strawberry gingivitis and nasal crusts (c, arrows)

Full size image

GPA is a systemic necrotizing granulomatous vasculitis of small vessels, strongly associated with PR3-ANCA, and commonly presenting with ear-nose-throat, respiratory tract, and renal involvements. About 15% of the patients also exhibit ocular involvement at diagnosis, possibly affecting all the ocular structures, including the eyelids [1, 2]. To our knowledge, only one other case of eyelid ulceration as initial presentation of GPA has been described [3].

To conclude, ulceration and even more perforation of eyelid are rare manifestations of GPA. Nonetheless, the rarity of this clinical sign should in our patients, as for perforation of the nasal septum, lead to the research of ANCA-associated vasculitis.