Sweet's Syndrome

  • AKA acute febrile neutrophilic dermatosis

 

  • Illness script: inflammatory condition (with pathogenesis to be definitively determined) presenting with an abrupt onset of tender erythematous skin lesions (papules, plaques, and nodules--often with pathergy), recurrent fevers >38 C, and often malaise and joint pain with laboratory findings of peripheral neutrophilia and elevated inflammatory markers and findings on skin biopsy of neutrophilic infiltration of the skin (in the upper dermis--without vasculitis).
  • Extracutaneous manifestations can occur with sterile neutrophilic inflammation in the bones, intestines, liver, aorta, lungs, and muscles.

 

  • Three types: classical (often preceded by URI, can be associated with IBD or pregnancy), malignancy-associated (most common association with AML or paraneoplastic; can precede, follow, or appear concurrent with the diagnosis of the patient's neoplasm!), or drug-induced (most common is granulocyte-stimulating factor).

 

  • Systemic corticosteroids are the therapeutic gold standard--often with prompt, dramatic response with improvement of skin lesions and constitutional symptoms.