What is the recommended treatment approach for Sweet syndrome in pediatric patients?

Sweet Syndrome in Children: Treatment Approach

First-Line Treatment

Systemic corticosteroids with prednisone at 1 mg/kg/day are the gold standard treatment for pediatric Sweet syndrome, providing rapid and dramatic improvement of symptoms and skin lesions within days. 1

• Prednisone should be administered as a single daily oral dose at 1 mg/kg/day, which typically results in resolution of fever and skin lesions within 48-72 hours 2, 3
• Treatment duration is typically 4-6 weeks with gradual tapering to prevent relapse 3, 4
• The excellent response to corticosteroids is so characteristic that it can serve as a diagnostic criterion for Sweet syndrome 5

Alternative and Second-Line Therapies

For patients in whom corticosteroids are contraindicated (such as those with systemic infections) or who experience recurrences, the following options should be considered:

• Potassium iodide (saturated solution) is highly effective as first-line alternative therapy, producing rapid resolution of symptoms and lesions 3, 4
• Colchicine can be used as monotherapy or adjuvant therapy, with similarly rapid response rates 4
• Dapsone has proven effective for recurrent cases, though requires careful monitoring for hemolytic anemia and methemoglobinemia 3, 4
• Indomethacin may be considered but appears less effective than corticosteroids, potassium iodide, or colchicine 4

Adjunctive Treatment Options

• Topical or intralesional corticosteroids can be effective as monotherapy for localized disease or as adjuvant therapy with systemic treatment 4
• Systemic antibiotics with anti-staphylococcal activity should be added when lesions are impetiginized or secondarily infected 4

Essential Diagnostic Workup

Before initiating treatment, perform comprehensive evaluation to identify underlying triggers:

• Hematologic malignancy screening is critical, as malignancy-associated Sweet syndrome occurs in pediatric patients, though less frequently than infection-associated cases 3, 6
• Infectious workup should be thorough, as infections are the most common pediatric trigger (more so than in adults) 3, 6
• Medication review to identify potential drug-induced cases, particularly all-trans retinoic acid and other medications 3, 4
• Immunodeficiency evaluation should be considered in appropriate clinical contexts 2

Monitoring and Follow-Up

• Monitor for recurrence, which occurs in approximately 40% of pediatric cases despite initial excellent response 3, 6
• Track inflammatory markers (CRP, neutrophil count) to assess treatment response 2, 3
• Evaluate for extracutaneous manifestations including arthralgias, ocular involvement, and organ involvement 5, 4

Important Clinical Pitfalls

• Do not delay treatment while awaiting biopsy results if clinical suspicion is high, as the condition causes significant morbidity from pain and fever 2, 6
• Do not assume idiopathic disease without thorough workup—underlying conditions determine prognosis and require specific management 6
• Spontaneous resolution can occur in some cases, particularly drug-induced Sweet syndrome after medication withdrawal, so consider observation in mild cases where the trigger has been removed 4
• Relapse is common even with appropriate initial treatment, so prepare families for potential need for second-line agents 3, 6