ABSTRACT
Fever Blood neutrophilic leukocytosis Raised painful plaques on the limbs, face, and neck Histologic findings of dense infiltration of the dermis by mature neutrophils
• The syndrome may be idiopathic, drug-related, or associated with malignancy
• In up to 20% of cases, the syndrome is associated with hematologic illness; of these, 50% are acute myelogenous leukemia
• Extracutaneous manifestations include diffuse arthralgias, proteinuria, and episcleritis
• Appropriate management includes treatment of the underlying disorder and use of systemic corticosteroids
• Potassium iodide and colchicine may be used in patients who should not take corticosteroids
