HemeOnc

Sweet Syndrome

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Sweet Syndrome, Sweet's Syndrome, Acute Neutrophilic Febrile Dermatosis

  • Epidemiology
  1. Age: 30-60 years old
  2. Gender: Female predominance by 3 to 1 ratio
  • Pathophysiology
  1. Unknown cause
  2. First case described in 1964 in a middle aged woman post-URI and with red Plaques and fever
  • Findings
  1. Systemic symptoms in 75% of patients
    1. Fever
    2. Malaise
    3. Myalgias
    4. Arthritis or Arthralgias
  2. Rash
    1. Non-pruritic rash (but may burn)
    2. Red edematous Plaques on the face, neck , upper trunk and extremities
  • Associated Conditions
  1. Classic: Preceded by infection (71% of cases)
    1. Preceding Upper Respiratory Infection
    2. Preceding gastrointestinal infection
  2. Malignancy (11% of cases)
    1. Acute myelogenous infection
    2. Solid tumors (less common)
      1. Genitourinary cancer
      2. Gastrointestinal cancer
      3. Breast Cancer
  3. Inflammatory conditions (16% of cases)
    1. Most cases are idiopathic
    2. Inflammatory Bowel Disease
  4. Pregnancy (2% of cases)
  5. Medications
    1. Granulocyte Colony Stimulating Factor
    2. Oral Contraceptive
    3. Trimethoprim-Sulfamethoxazole
    4. Minocycline
    5. Furosemide
  • Diagnosis
  1. Major criteria (both required)
    1. Red edematous Plaques
    2. Classic biopsy findings
      1. Neutrophils present
      2. Karorrhexis
      3. Papillary dermal edema
  2. Minor criteria (2 required)
    1. Identified trigger event
      1. Preceding respiratory infection
      2. Preceding gastrointestinal infection
      3. Preceding Immunization
      4. Inflammatory disease
      5. Myeloproliferative disease
      6. Acute infection
      7. Pregnancy
    2. Fever and Malaise
    3. Lab markers of inflammation
      1. Erythrocyte Sedimentation Rate >20
      2. C-Reactive Protein increased
      3. Leukocytosis with Left Shift
    4. Significant improvement on Corticosteroids
  • Evaluation
  • Secondary cause search indications
  1. Age over 50
  2. Anemia
    1. Seen in more than 70% of cancer associated with Sweet Syndrome
  3. Thrombocytopenia
    1. Seen in 50% of cancer associated with Sweet Syndrome
  4. Bullous lesions or necrosis
  5. Solitary or ulcerative lesions
    1. More commonly associated with cancer
  • Management
  1. Systemic Corticosteroids