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