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

Sweet Syndrome in Children: Treatment Approach

First-Line Treatment

Systemic corticosteroids are the gold standard treatment for pediatric Sweet syndrome, with prednisone at 1 mg/kg/day providing rapid and dramatic improvement of symptoms and skin lesions within days. 1, 2, 3, 4

• Prednisone should be initiated at 1 mg/kg/day as a single daily dose, with most pediatric cases showing excellent response within 48-72 hours 2, 3
• Treatment duration typically ranges from 2-6 weeks with gradual tapering based on clinical response 4, 5
• Response is characterized by rapid resolution of fever, improvement in systemic inflammation markers (CRP, neutrophil count), and healing of skin lesions 2, 3

Clinical Presentation to Recognize

Sweet syndrome in children presents with:

• Tender erythematous-violaceous plaques, nodules, or papules most commonly on face, neck, upper limbs, and back 2, 3, 6
• High fever (often persistent despite antibiotics if infection was initially suspected) 2, 3
• Marked neutrophilia (absolute neutrophil counts often >10,000/mm³, sometimes exceeding 40,000/mm³) 3
• Elevated inflammatory markers (CRP typically >100 mg/L) 3
• Histopathology showing dense dermal neutrophilic infiltrate without vasculitis 2, 4, 5

Essential Workup Before Treatment

Before initiating corticosteroids, evaluate for underlying triggers:

• Screen for infections (particularly Mycoplasma pneumoniae, upper respiratory infections, and other bacterial/viral pathogens) as infection is the most common trigger in pediatric cases 2, 6
• Evaluate for hematologic malignancy with complete blood count with differential, peripheral smear, and consider bone marrow evaluation if concerning features present 2, 3, 6
• Review medication history for drug-induced cases (though less common in children than adults) 4, 5
• Consider immunodeficiency screening in recurrent cases or those with complex medical history 3, 6

Alternative and Second-Line Therapies

For patients with contraindications to corticosteroids or recurrent disease:

Potassium Iodide

• Saturated solution of potassium iodide (SSKI) is highly effective as first-line alternative therapy 2, 4, 5
• Provides rapid resolution comparable to corticosteroids 4, 5
• Particularly useful when systemic infection is suspected or corticosteroids are contraindicated 4

Dapsone

• Effective for recurrent cases or as steroid-sparing agent 2, 4, 5
• Requires G6PD screening before initiation and monitoring for methemoglobinemia and hemolytic anemia 4
• Typical dosing considerations require hematologic monitoring 4

Colchicine

• Alternative first-line oral agent with rapid response 4, 5
• May be preferred in patients requiring long-term management 4

Other Options

• Indomethacin (less effective than above agents) 4, 5
• Cyclosporine (requires careful monitoring for nephrotoxicity and hypertension) 4, 5
• Clofazimine (less effective, reserved for refractory cases) 4, 5

Management of Infection-Associated Cases

When Sweet syndrome is triggered by infection:

• Treat the underlying infection with appropriate antimicrobials 4, 6
• Corticosteroids can be initiated concurrently with antimicrobial therapy 4
• Some infection-associated cases may show partial improvement with antibiotics alone, particularly if secondary impetiginization is present 4

Prognosis and Recurrence Management

• Prognosis is generally excellent in pediatric cases 2, 6
• Recurrences occur in approximately 30-40% of cases 2, 5
• For recurrent disease, consider:
  • Longer corticosteroid taper 5
  • Addition of steroid-sparing agents (dapsone, potassium iodide, or colchicine) 2, 4
  • Re-evaluation for underlying triggers including occult malignancy 6, 5

Critical Pitfalls to Avoid

• Do not mistake Sweet syndrome for bullous impetigo or other infectious processes—this delays appropriate treatment and prolongs morbidity 3
• Do not withhold corticosteroids while awaiting biopsy results if clinical suspicion is high and patient is systemically ill 2, 3
• Do not assume idiopathic disease without thorough evaluation for infection and malignancy, as these are more common triggers in children than adults 2, 6
• Do not discontinue treatment prematurely—adequate treatment duration prevents early recurrence 4, 5

Special Considerations

• Spontaneous resolution can occur without treatment in some cases, but this is unpredictable and patients often have significant morbidity from symptoms 4, 5
• Drug-induced cases may resolve with medication withdrawal alone, though corticosteroids accelerate resolution 4, 5
• In malignancy-associated cases, treatment of the underlying cancer is essential, though corticosteroids remain necessary for symptom control 4, 5