What is the recommended treatment approach for an 8-year-old patient with suspected Sweet syndrome, considering potential underlying conditions and systemic involvement?

Sweet Syndrome in an 8-Year-Old: Treatment Approach

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

Initial Assessment and Workup

Before initiating treatment, evaluate for underlying conditions that commonly trigger Sweet syndrome in children:

• Screen for infections (upper respiratory tract infections, gastrointestinal infections) as these are more frequently associated with pediatric Sweet syndrome than malignancies 2
• Assess for hematologic malignancy through complete blood count with differential, peripheral blood smear, and bone marrow evaluation if anemia or other cytopenias are present 2, 3
• Document baseline inflammatory markers including neutrophil count, ESR, and CRP 4, 5
• Evaluate for drug exposure including recent medications, particularly if the patient has an underlying condition requiring treatment 6, 2

First-Line Treatment Protocol

Prednisone 1 mg/kg/day orally as a single daily dose is the recommended initial therapy 1:

• Expect rapid clinical response within 24-72 hours with improvement in fever, systemic symptoms, and skin lesions 4, 5
• Continue treatment for 4-6 weeks at full dose, then taper gradually over 2-5 months 7
• Monitor for treatment response by assessing resolution of fever, improvement in skin lesions, and normalization of inflammatory markers 3

Alternative First-Line Options

If systemic corticosteroids are contraindicated (e.g., active infection, immunocompromised state):

• Potassium iodide provides rapid resolution of symptoms and lesions, making it reasonable as first-line therapy when corticosteroids cannot be used 4, 5
• Colchicine similarly results in rapid resolution and can be used as first-line therapy in patients with contraindications to corticosteroids 4, 5

Adjunctive Therapies for Localized Disease

• Topical high-potency corticosteroids or intralesional corticosteroids may be effective for localized lesions, either as monotherapy for limited disease or as adjuvant therapy 4, 5

Second-Line Treatment for Recurrent or Refractory Disease

Approximately one-third of pediatric patients experience recurrence after initial treatment 2:

• Dapsone has been successfully used in pediatric patients with recurrent Sweet syndrome 2
• Saturated solution of potassium iodide (SSKI) is effective for recurrent cases 2
• Indomethacin may be considered but appears less effective than corticosteroids, potassium iodide, or colchicine 4
• Cyclosporine can be effective but requires careful monitoring for adverse effects 4, 5

Treatment of Underlying Conditions

• If infection-associated: Treat the underlying infection with appropriate antimicrobials; spontaneous resolution may occur after infection clearance 5, 2
• If malignancy-associated: Coordinate treatment with oncology; the dermatosis often improves with treatment of the underlying malignancy 6, 3
• If drug-induced: Withdraw the offending medication; spontaneous resolution may occur without additional intervention 4, 5

Monitoring and Follow-Up

• Assess treatment response by monitoring fever resolution, improvement in skin lesions, and normalization of neutrophil count and inflammatory markers 3
• Screen for recurrence as pediatric Sweet syndrome can recur even after successful initial treatment 2
• Monitor for underlying malignancy particularly if anemia is present, as this is significantly associated with malignancy-associated Sweet syndrome 3

Critical Pitfalls to Avoid

• Do not delay corticosteroid initiation while awaiting complete workup in symptomatic patients, as treatment provides rapid relief and does not interfere with diagnostic evaluation 4, 5
• Do not assume infection-associated Sweet syndrome will resolve spontaneously; while this can occur, most pediatric patients require systemic treatment 2
• Do not overlook hematologic malignancy screening, particularly in patients with anemia, as approximately 21% of Sweet syndrome cases are malignancy-associated 6, 3
• Do not use dapsone or cyclosporine as first-line therapy without appropriate baseline laboratory monitoring (G6PD for dapsone, renal function and blood pressure for cyclosporine) due to potential severe adverse effects 4