A 49-year-old woman with edema localized to the right eyelid for 3 weeks visited our hospital (Picture A). She had had allergic rhinitis and sinusitis for 10 years and bronchial asthma for 5 years. She was being treated with nasal steroids and high-dose inhaled corticosteroids. She had no fever, arthralgia, purpura, or peripheral neuropathy. Computed tomography revealed ethmoiditis and consolidation with ground-glass opacities in the right lung. Laboratory investigations revealed an elevated peripheral blood eosinophil count of 2,142 /μL, non-specific IgE level of 640 IU/mL (reference range, ≤173 IU/mL), and rheumatoid factor level of 32 IU/mL (reference range, ≤15 IU/mL). Systemic inflammatory markers, including C-reactive protein (0.06 mg/dL), myeloperoxidase-antineutrophil cytoplasmic antibody, and proteinase 3 antineutrophil cytoplasmic antibody, were negative. A right upper eyelid skin biopsy was performed, which revealed subcutaneous tissue and perivascular eosinophilic infiltration with granuloma (blue arrowhead) and enlarged vascular endothelial cells (yellow arrowhead) (Picture B). We diagnosed her with eosinophilic granulomatosis with polyangiitis (EGPA) based on the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for EGPA [total of 13 points: small vasculitis in the eyelids (≥6 points), obstructive airway lesions (3 points), eosinophilic inflammation outside blood vessels (2 points), and eosinophil count 1×109/L (5 points)] (1). Prednisolone therapy was initiated, and the eyelid edema resolved. Angioedema and dermatomyositis are the differential diagnoses of eyelid edema, but the presence of granuloma and the absence of lymphocytic inflammatory cell infiltrate in this case favor the diagnosis of EGPA. Clinicians should consider EGPA in patients allergic to eyelid edema.
Picture.
Informed consent was obtained from the patient to report this case.
The authors state that they have no Conflict of Interest (COI).
References
- 1.Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis 81: 309-314, 2022. [DOI] [PubMed] [Google Scholar]