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Therapeutic Strategies

Sweet's Syndrome (Acute Febrile Neutrophil Dermatosis)

Timothy Berger Bruce Wintroub

Saturday, January 01, 2011

First Steps

  1. Oral prednisone 40-60 mg/day will result in a dramatic clinical response in 24-48 hours.
  2. Continue prednisone at the initial dose until lesions have completely or almost completely resolved.

Subsequent Steps

  1. After response, reduce prednisone by 10-mg increments each week over the next 4-6 weeks to the dose necessary to control the disorder. Most patients will be completely weaned from steroids in 6-8 weeks.
  2. Relapse may require reinstitution of steroids and another attempt at tapering medication to the control dose.
  3. Patients requiring long-term systemic steroids are most safely treated with alternate day therapy.
  4. In severe or refractory disease cyclosporine A, 5-10 mg/kg per day may induce a response.
  5. Infliximab 5 mg/kg/day at weeks 0, 2, and 6 or etanercept 50 mg twice weekly may be considered as rescue therapy in patients failing the above treatments.

Alternative Steps

  1. In mild cases, dapsone 100-200 mg/day may be effective.
  2. Colchicine 0.6-1.2 mg once to twice daily (as tolerated due to the diarrhea) may be used as a steroid-sparing agent or in mild cases as a solitary agent.

Pitfalls

  1. Sweet's syndrome may be associated with acute myelogenous leukemia, and may present during a leukemic or preleukemic state. These patients are frequently anemic.
  2. Granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy may be complicated by Sweet's syndrome.

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