Treatment of Sweet's Syndrome
Systemic corticosteroids are the gold standard first-line treatment for Sweet's syndrome, with rapid resolution of symptoms typically occurring within 48-72 hours of initiation. 1, 2, 3
Clinical Presentation and Diagnosis
Sweet's syndrome (acute febrile neutrophilic dermatosis) is characterized by:
- Tender, red inflammatory nodules or papules, usually affecting the upper limbs, face, or neck
- Fever
- Neutrophilia
- Histopathology showing neutrophilic infiltrates in the dermis without vasculitis
Sweet's syndrome can be classified into three types:
- Classical/idiopathic Sweet's syndrome
- Malignancy-associated Sweet's syndrome
- Drug-induced Sweet's syndrome
Treatment Algorithm
First-Line Therapy:
- Systemic corticosteroids: Prednisone 0.5-1.0 mg/kg/day 2, 3, 4
- Typical duration: 4-6 weeks with gradual tapering
- Rapid response usually occurs within 48-72 hours
- Complete resolution of lesions typically within 1-2 weeks
Alternative First-Line Options (for patients with contraindications to corticosteroids):
- Potassium iodide: 300-900 mg/day divided into three doses 2, 3
- Colchicine: 0.5 mg 2-3 times daily 2, 3, 4
Second-Line Therapies:
- Dapsone: 100-200 mg/day (requires G6PD screening before initiation) 2, 3
- Cyclosporine: 2-5 mg/kg/day 2, 3
- Indomethacin: 150 mg/day in divided doses 2, 3
- Clofazimine: 100-200 mg/day 2
For Localized Lesions:
Special Considerations
Associated Conditions
- Sweet's syndrome is frequently associated with inflammatory bowel disease, particularly ulcerative colitis 1, 5
- Approximately 35% of cases are associated with malignancy, most commonly acute myelogenous leukemia 3, 6
- Patients with laboratory evidence of anemia should be evaluated for underlying malignancy 6
Recurrence
- Approximately one-third of patients with classical Sweet's syndrome experience recurrence 3
- For recurrent cases, maintenance therapy with one of the steroid-sparing agents may be considered
Monitoring
- Monitor for secondary infection of lesions
- Regular assessment of wound healing progress
- Appropriate laboratory monitoring when using dapsone or cyclosporine due to potential adverse effects
Evidence Quality and Limitations
The evidence for systemic corticosteroids as first-line therapy is robust and consistent across multiple studies and guidelines. However, most of the evidence for alternative therapies comes from case reports and small case series rather than randomized controlled trials.
The European evidence-based consensus on ulcerative colitis specifically mentions that Sweet's syndrome is effectively treated with systemic corticosteroids 1, and multiple reviews confirm this as the gold standard therapy 2, 3, 4, 6.
For patients who cannot tolerate corticosteroids or have contraindications, potassium iodide and colchicine have shown good efficacy as alternative first-line agents 2, 3.