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- Presentation
Periorbital Inflammatory and Infectious Lesions: Sweet Syndrome, Lupus, and EBV-Positive Mucocutaneous Ulcer
Description
The presentation highlighted three periorbital disease cases that were initially concerning for infection but had different diagnoses. First, a neutropenic patient with relapsed AML developed rapidly progressive eyelid swelling, bruising, and lesions on the extremities; biopsy showed dermal inflammation with neutrophils, edema, hemorrhage, and no organisms, leading to a diagnosis of Sweet syndrome with ocular involvement that improved dramatically after systemic prednisone. Second, a woman with antiphospholipid syndrome had worsening eyelid edema and violaceous plaques that failed antibiotic treatment; biopsy of a non-ocular lesion showed vacuolar interface dermatitis, thickened basement membrane, and dermal mucin consistent with cutaneous lupus, and she improved quickly with IV steroids, illustrating that lupus can present as periorbital swelling and mimic cellulitis. Third, an elderly woman on methotrexate for rheumatoid arthritis had a chronic ulcerated eyelid nodule; biopsy revealed branching septate hyphae and cultures grew Purpureocillium lilacinum, but the dense lymphoplasmacytic infiltrate also showed EBV-positive B-cell proliferation consistent with EBV-positive mucocutaneous ulcer. The case emphasized that this EBV-driven lesion often occurs in immunosuppressed patients, can coexist with infection, and may resolve after stopping immunosuppression, as it did in this patient.
View moreConclusions
- Periorbital swelling and bruising in immunocompromised patients can be caused by inflammatory dermatoses such as Sweet syndrome rather than infection, and recognition is critical because corticosteroids can produce rapid resolution and protect vision.
- Periorbital edema may be an uncommon presenting sign of systemic lupus erythematosus, is often mistaken for cellulitis or angioedema, and typically improves quickly with lupus-directed therapy such as systemic steroids and hydroxychloroquine.
- Biopsy of atypical eyelid lesions can reveal the true diagnosis when clinical appearance is misleading, helping distinguish inflammatory disease from infection or malignancy.
- In immunosuppressed patients, chronic eyelid ulcers may represent a dual process, including both opportunistic fungal infection and an underlying EBV-positive mucocutaneous ulcer.
- Purpureocillium lilacinum should be considered an emerging ocular-cutaneous pathogen in immunocompromised hosts and may require newer azole therapy because it is often resistant to many standard antifungals.
- EBV-positive mucocutaneous ulcer is a localized B-cell lymphoproliferative disorder associated with immune suppression that generally has a favorable prognosis and often resolves after stopping the offending immunosuppressive agent.
- Concomitant infection may contribute to local immune dysregulation and can coexist with or unmask EBV-positive mucocutaneous ulcer.
- Overall, careful clinicopathologic correlation and early biopsy are essential for diagnosing unusual periorbital lesions because several distinct diseases can mimic cellulitis but require very different treatments.
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