The New England Journal of MedicineCase 22-2024: A 30-Year-Old Woman with Postpartum Fever, Abdominal Pain, and Skin Ulcers

Postpartum Sweet’s Syndrome: A Rare but Serious Neutrophilic Dermatosis with Systemic Manifestations

A 30-year-old woman developed fever, abdominal pain, and skin ulcers 6 days after delivering her first child at 35 weeks gestation. Initially treated for suspected endometritis, her condition worsened despite broad-spectrum antibiotics. The case highlights the challenges in diagnosing Sweet’s syndrome, a rare neutrophilic dermatosis that can mimic infection and have severe systemic manifestations.

Key Points:

• The patient presented with fever, abdominal pain, and leukocytosis 6 days postpartum.
• Initial diagnosis was endometritis, but symptoms persisted despite antibiotic therapy.
• Imaging revealed intra-abdominal fluid collections, ascites, and anasarca.
• Skin lesions developed, including ulcerating plaques at sites of minor trauma (pathergy).
• Skin biopsy showed dense neutrophilic infiltrate without vasculitis, consistent with Sweet’s syndrome.
• Diagnosis of Sweet’s syndrome was based on major and minor criteria, including typical skin lesions, histopathology, fever, and elevated inflammatory markers.
• Pregnancy-associated Sweet’s syndrome is rare, accounting for only 2% of cases.
• Systemic manifestations included possible pulmonary involvement and right heart strain.
• Treatment with high-dose glucocorticoids led to rapid improvement within 12 hours.
• Sweet’s syndrome can recur in subsequent pregnancies but overall has favorable perinatal outcomes.
• Differential diagnosis included endometritis, pyogenic infections, and other neutrophilic dermatoses.
• The patient’s history of hidradenitis suppurativa and family history of inflammatory conditions may have been relevant risk factors.
• Timely diagnosis and treatment are crucial to prevent progression to severe organ involvement.
• Sweet’s syndrome can be associated with malignancy, infections, drugs, or inflammatory conditions.
• First-line treatments include systemic glucocorticoids, dapsone, potassium iodide, and colchicine.

Classical Sweet syndrome in adults affects women more often than men by as much as 15:1 by some estimates. This female preponderance has not been seen in malignancy-associated or drug-induced Sweet syndrome. Classical Sweet syndrome usually affects women between the ages of 30-50, but can be seen in individuals of any age including children. (NORD)

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