What is the treatment for Sweet syndrome?

Treatment of Sweet Syndrome

Systemic corticosteroids are the gold standard treatment for Sweet syndrome, with prednisone 1 mg/kg/day (maximum 60 mg daily) for 2-4 weeks followed by a gradual taper being the recommended first-line therapy. 1

First-Line Treatment

Systemic Corticosteroids

• Dosage: Prednisone 1 mg/kg/day (typically 40-60 mg daily) as a single morning dose 1
• Duration: 2-4 weeks followed by a gradual taper over a similar period 1, 2
• Expected response: Dramatic improvement of both symptoms and skin lesions within 48-72 hours 3
• Monitoring: Watch for corticosteroid side effects, especially in patients with diabetes, hypertension, or osteoporosis

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

1. Potassium iodide

   • Rapidly effective alternative when corticosteroids are contraindicated 2
   • Typically administered as saturated solution (SSKI) or tablets
   • Onset of action is typically within 24-48 hours

2. Colchicine

   • Dosage: 0.5 mg 2-3 times daily
   • Particularly useful when corticosteroids are contraindicated 2, 3
   • Monitor for GI side effects (diarrhea, nausea)

Second-Line Treatment Options

For refractory cases or when first-line therapies are contraindicated:

1. Indomethacin

   • Dosage: 150 mg/day for the first week, then 100 mg/day for two additional weeks 4
   • Effective in approximately 95% of patients with fewer relapses compared to corticosteroids 4
   • Monitor for GI side effects and avoid in patients with peptic ulcer disease

2. Dapsone

   • Requires baseline G6PD screening and regular blood monitoring
   • Less effective than corticosteroids, potassium iodide, or colchicine 2

3. Cyclosporine

   • Reserved for severe or refractory cases
   • Requires careful monitoring of renal function and blood pressure 2

4. Clofazimine

   • Less commonly used due to availability issues and skin discoloration
   • Generally less effective than other options 3

Treatment Based on Clinical Subtype

Classical (Idiopathic) Sweet Syndrome

• Follow standard treatment algorithm with systemic corticosteroids as first-line therapy
• Consider potassium iodide or colchicine if corticosteroids are contraindicated

Malignancy-Associated Sweet Syndrome

• Treat the underlying malignancy when possible
• Systemic corticosteroids for acute management of symptoms
• May require more prolonged therapy or higher doses

Drug-Induced Sweet Syndrome

• Discontinue the offending medication
• Systemic corticosteroids for symptomatic relief
• Symptoms may resolve spontaneously after drug withdrawal 3

Localized Disease

• For limited cutaneous involvement, consider:
  • High-potency topical corticosteroids
  • Intralesional corticosteroid injections
  • These may be used as monotherapy or as adjuncts to systemic treatment 2

Management of Recurrent Disease

• Approximately one-third of patients with classical Sweet syndrome experience recurrence 3
• For frequent relapses, consider:
  • Low-dose maintenance therapy with colchicine
  • Dapsone as a steroid-sparing agent
  • Investigation for underlying triggers or associated conditions

Special Considerations

• In patients with diabetes: Monitor blood glucose closely when using systemic corticosteroids
• In pregnant patients: Corticosteroids are generally considered safer than other options
• In patients with systemic infections: Prefer potassium iodide or colchicine over corticosteroids 2

Emerging Therapies

Recent evidence suggests potential efficacy of biologics targeting IL-1, IL-17, and TNF-α pathways in refractory cases, though these should be considered experimental and used only when standard therapies fail 5.