SHM Converge Scientific Abstract ePoster Gallery-Necrotizing Sweet Syndrome, a Not-So-Sweet Post-Operative Complication

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Necrotizing Sweet Syndrome (NSS), also called acute febrile neutrophilic dermatosis, is a rare inflammatory skin condition first described in 1964. It presents with fever, leukocytosis, and painful erythematous or edematous plaques, usually without active infection. NSS is uncommon, affects mostly women aged 30–60, and may be associated with autoimmune disease, malignancy, or be idiopathic. Because it can resemble necrotizing fasciitis, distinguishing the two is critical: fasciitis requires surgery and antibiotics, while NSS is treated primarily with corticosteroids. Misdiagnosis and unnecessary surgery can worsen NSS lesions.<br /><br />Diagnosis is based on clinical features and biopsy. Current criteria require two major and two minor findings. Histology is a major criterion, making biopsy essential, though diagnosis can be difficult because biopsy may not always show neutrophil predominance; lymphocytic or eosinophilic infiltrates can also occur. Epidermal sparing, spongiosis, ulceration, necrosis, and subcorneal pustules may be seen. Pathogenesis is not fully understood but likely involves immune and genetic factors.<br /><br />In the case presented, a 5-year-old boy developed worsening drainage, fever, and inflammation one week after bilateral hip replacement. He initially met sepsis criteria and was treated for possible infection with broad-spectrum antibiotics, but cultures were negative and symptoms persisted. Dermatology suspected neutrophilic dermatosis/NSS due to the postoperative setting, fever, leukocytosis, elevated ESR/CRP, and wound appearance. Biopsy showed ulceration, necrosis, marked acute inflammation, and spongiosis without neutrophilic predominance. High-dose prednisone led to clinical improvement, with decreased drainage and fever.<br /><br />First-line treatment for NSS is oral corticosteroids, typically prednisone 1 mg/kg/day or 40–60 mg, tapered over 4–6 weeks. Alternatives for steroid intolerance include colchicine or potassium iodide. Recurrent cases may require dapsone, indomethacin, clofazimine, cyclosporine, or potassium iodide.

Asset Subtitle

Mariam Ansar

Meta Tag

Author List Aakhila Rameeza, Kathryn Haroldson, Kimberly Tena Diaz, Mariam Ansar

Category Clinical Vignettes

Concept Necrotizing Sweet Syndrome

Concept Diagnostic Criteria

Concept Systemic Corticosteroid

Concept Acute Febrile Neutrophilic Dermatosis

Concept Clinical Presentation

Distinguished Non-Finalist

Presenter Organization Cooper University Hospital

Presenting Author Mariam Ansar

Track Adult

Keywords

Necrotizing Sweet Syndrome

acute febrile neutrophilic dermatosis

corticosteroid treatment

necrotizing fasciitis differential

skin biopsy diagnosis

fever and leukocytosis

neutrophilic dermatosis

postoperative inflammation

prednisone therapy

autoimmune malignancy association

Necrotizing Sweet Syndrome

Diagnostic Criteria

Systemic Corticosteroid

Acute Febrile Neutrophilic Dermatosis

Clinical Presentation