Abstract ID: 26-399
Acute febrile neutrophilic dermatosis: A rare case of Sweet syndrome masquerading as preseptal cellulitis
Author: Yasaman Ataei Base Hospital / Institution: University of Pennsylvania/Children’s Hospital of Philadelphia (CHOP)
Presentation Type: ePoster Presentation
Purpose
Sweet syndrome is a rare inflammatory condition characterized by tender erythematous papules, fever, and elevated inflammatory markers, and neutropenia most commonly associated with myeloid leukemia (AML) and myelodysplastic syndrome (MDS). Its hallmark histopathological finding is a dense dermal neutrophilic infiltrate without leukocytoclastic vasculitis. Here we present a rare case of sweet syndrome masquerading as periorbital cellulitis.
Methods
Case Report.
Results
A 60-year-old female with relapsed TP53-mutated AML presented with neutropenic fever and left eyelid edema and erythema concerning for preseptal cellulitis. BCVA was 20/25 OD and 20/50 OS. External exam showed left upper eyelid crusty erythema, anterior segment exam revealed superficial punctate keratitis OS, and CT imaging confirmed preseptal soft-tissue swelling without abscess. She was admitted and started on vancomycin and cefepime.
Over the following week, cellulitis spread to both eyelids and the right face. By day 6, BCVA declined to 20/400 OS with a new conjunctival membrane and dense Descemet folds OS appeared. The patient also developed indurated targetoid plaques on the extremities and pathergy at IV sites. Despite antibiotics, febrile neutropenia persisted without an identifiable organism. Dermatology and otolaryngology were consulted, and Amphotericin B was added given concern for angioinvasive fungal infection.
By day 7, BCVA declined to CF OS. Nasal endoscopy and MRI orbits were negative for fungal sinusitis. Dermatology biopsied the targetoid lesions and raised concern for Sweet syndrome given the constellation of findings. The patient was empirically started on prednisone 1 mg/kg/day.
After 6 days of steroids, facial erythema nearly resolved, BCVA improved to 20/20 OU, and Descemet folds fully resolved. Pathology confirmed dense dermal neutrophilic infiltrate without vasculitis or leukemia cutis, and all cultures remained negative.
Conclusion
This case highlights the importance of considering Sweet syndrome in AML patients with an atypical clinical course or poor response to antimicrobial treatment.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Victor | Cox | University of Pennsylvania |
| Makayla | McCoskey | University of Pennsylvania |