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Sweet’s Syndrome (Acute Febrile Neutrophil Dermatosis)

Clinical Case

Case 1

  • 53-year-old female
  • No significant past medical history and she takes no medications
  • Review of systems is notable only for recent fevers in association with the rash
  • Presents for management of painful nodules on the legs and arms

Initial evaluation

  • Tired-appearing female
  • Low-grade fever is noted
  • Juicy indurated erythematous nodules on the arms and legs, few scattered on trunk without mucosal lesions
  • Diagnosis: favor Sweet’s syndrome
  • A skin biopsy to confirm the diagnosis is performed.
  • Concern for an underlying inflammatory condition or malignancy triggers a diagnostic evaluation including age-appropriate cancer screening, chest X-ray, and blood tests including complete blood count with differential, erythrocyte sedimentation rate, and rheumatologic serologies; given the absence of gastrointestinal symptoms, a colonoscopy to rule out inflammatory bowel disease is deferred
  • Follow-up in 1 week

Follow-up evaluation

  • The rash has not progressed but also shows no improvement
  • Blood tests indicate a mild anemia and thrombocytopenia and slightly elevated erythrocyte sedimentation rate; the remainder of her tests and imaging are normal
  • Given the high suspicion for idiopathic Sweet’s syndrome, the patient is started on systemic prednisone 1 mg/kg daily and given instructions to take vitamin D and calcium supplements
  • Follow-up in 1 week

Follow-up evaluation

  • Patient reports significant clinical improvement within 48 hours of starting prednisone
  • The patient’s prednisone is tapered over the next 6 weeks with recurrence of symptoms; after escalation of prednisone dose, her symptoms remit; dapsone is started, and her prednisone is successfully tapered off.
  • The patient remains stable on dapsone