What is the best treatment approach for Sweet's syndrome?

Treatment Approach for Sweet's Syndrome

Systemic corticosteroids are the gold standard treatment for Sweet's syndrome, with prompt response typically seen within 48-72 hours of initiation. 1, 2

First-Line Treatment Options

Systemic Corticosteroids

• Oral prednisone/prednisolone: 0.5-1 mg/kg/day as a single daily dose
• Continue until clinical resolution (typically 2-4 weeks)
• Taper gradually to prevent relapse
• Expected response: Dramatic improvement in both symptoms and skin lesions within 48-72 hours

Alternative First-Line Options (for patients with contraindications to corticosteroids)

1. Potassium iodide:

   • 300-900 mg/day (divided doses)
   • Rapid resolution of symptoms and lesions
   • Monitor thyroid function

2. Colchicine:

   • 0.5-1.5 mg/day
   • Particularly effective for recurrent cases
   • Monitor for GI side effects

Second-Line Treatment Options

When first-line treatments fail or are contraindicated:

1. Indomethacin:

   • 150 mg/day for first week, then 100 mg/day for two additional weeks 3
   • Good response in 17 of 18 patients in one study
   • Monitor for GI side effects

2. Dapsone:

   • 100-200 mg/day
   • Requires baseline G6PD testing and regular blood monitoring

3. Cyclosporine:

   • 2-5 mg/kg/day in divided doses
   • Requires monitoring of renal function and blood pressure

4. Clofazimine:

   • 100-200 mg/day
   • Less effective than other options

Topical Therapy

• High-potency topical corticosteroids for localized lesions
• Can be used as monotherapy for mild cases or as adjunctive therapy

Special Considerations

Malignancy-Associated Sweet's Syndrome

• Treat underlying malignancy when present
• May require more aggressive or prolonged corticosteroid therapy
• Consider hematology/oncology consultation

Drug-Induced Sweet's Syndrome

• Discontinue the offending medication
• Symptoms may resolve spontaneously after drug withdrawal
• Short course of corticosteroids may accelerate resolution

Recurrent Sweet's Syndrome

• Consider maintenance therapy with colchicine or dapsone
• Evaluate for underlying conditions that may trigger recurrence

Treatment Algorithm

1. Confirm diagnosis (clinical presentation + skin biopsy showing neutrophilic infiltrate)
2. Evaluate for underlying causes:
   • Malignancy (particularly hematologic)
   • Medications
   • Inflammatory conditions
3. Initiate treatment:
   • If no contraindications: Systemic corticosteroids
   • If corticosteroids contraindicated: Potassium iodide or colchicine
4. Monitor response:
   • Expect improvement within 48-72 hours
   • If no response within 1 week, reevaluate diagnosis or consider alternative therapy
5. Taper treatment once resolution achieved
6. Follow-up to monitor for recurrence

Pitfalls and Caveats

• Failure to identify and address underlying triggers may lead to recurrence
• Inadequate duration of therapy or too-rapid tapering of corticosteroids may result in relapse
• Always consider infection in the differential diagnosis before initiating immunosuppressive therapy
• Some patients may experience spontaneous resolution without therapy, but treatment accelerates recovery and reduces symptoms

Sweet's syndrome typically responds well to appropriate therapy with complete resolution, though approximately one-third of patients with classical Sweet's syndrome may experience recurrence of the dermatosis 2.