Churg Strauss syndrome (CSS) was first described in 1951 by Churg and Strauss. Eye involvement, though rare, can present as episcleritis, uveitis, ischaemic optic neuropathy, and nerve palsies. We report a case of CSS presenting with dacryoadenitis.
A 27-year-old female presented with a 2-week history of a non-fluctuant, mobile, tender left supero-temporal orbital mass. Upper lid eversion revealed a subconjunctival lesion in the same region. Significant medical history included asthma, recent rhinitis, and a 12-month history of a generalized maculopapular rash. Rapid progression was noted over the next week with the development of a partial ptosis and extension of the subconjunctival lesion to the limbus (Figures 1 and 2). A CT scan showed an enlarged lacrimal gland, with no bony erosion (Figure 3). The patient underwent lacrimal gland, conjunctival and skin biopsies.
Histopathology confirmed the presence of granulomatous lesions from both eye sites. Skin biopsy showed similar lesions with extravascular eosinophilic infiltration (Figure 4). Peripheral blood eosinophil levels were raised (2.13 × 109/l). ANCA screen was positive for pANCA but further analysis showed normal PR3 and MPO.
After confirmation of diagnosis, the patient responded dramatically to oral prednisolone 40 mg/day on a tapering regimen (Figure 5).
Our case showed asthma, eosinophilia, paranasal sinus involvement, and eosinophilic infiltration on histopathology, meeting the diagnostic criteria of the College of Rheumatologists.1
Dacryoadenitis was diagnosed clinically and on CT imaging. Histopathology confirmed the diagnosis with classical granulomatous inflammation with eosinophilic infiltration. CT imaging, blood tests, and histopathology ruled out lacrimal gland tumour, Wegener's granulomatosis and sarcoidosis.
Takanashi et al2 reported two cases of CSS, one of which presented as chronic dacryoadenitis, the other with vasculitis. It has been proposed that CSS evolves through an allergic phase to eosinophilic tissue infiltration and then a final vasculitic stage.3 Indeed, various case reports4, 5 have reported ocular involvement from both ends of this disease spectrum.
Although CSS may have varied ophthalmic presentation, asthma, eosinophilia, and multisystem involvement should raise suspicion of this condition. Early diagnosis and treatment could avoid complications and relapses, not uncommon in CSS.
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None of the authors have any financial interest to declare.
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Khandwala, M., Vayalambrone, D., Ong, J. et al. Dacryoadenitis as a presenting feature of the Churg Strauss syndrome. Eye 24, 385–386 (2010). https://doi.org/10.1038/eye.2009.109