Sweet's syndrome

Last edited 08/2023 and last reviewed 11/2023

This condition was originally described by Dr. Robert Douglas Sweet in 1964

  • an acute febrile neutrophilic dermatosis
    • characterized by fever, neutrophilia, erythematous and tender skin lesions that typically show an upper dermal infiltrate of mature neutrophils, with prompt improvement after the initiation of treatment
      • lesions have a transparent, vesicle-like appearance secondary to the pronounced oedema in the upper dermis (pseudovesicular appearance)
      • clinical presentation is with acute, tender, erythematous plaques, pseudovesicles and, occasionally, blisters with an annular or arciform pattern
        • rash occurs on the head, neck, legs, and arms, particularly the back of the hands and fingers
          • trunk is rarely involved
      • histological findings of dense neutrophilic infiltrate without any evidence of primary vasculitis

  • three main clinical types which are described include: Classical or idiopathic SS, malignancy-associated or paraneoplastic SS, and drug-induced SS
    • Classical or idiopathic Sweet's syndrome may be associated with infection (upper respiratory tract or gastrointestinal tract), inflammatory bowel disease, or pregnancy

  • recurrent episodes of Sweet's syndrome occur in one-third to two-thirds of patients

  • Sweet's syndrome can also present as a paraneoplastic syndrome (most commonly related to acute myelogenous leukaemia) or as a medication-related disorder (most commonly after treatment with granulocyte-colony stimulating factor therapy)
    • approximately 70% of cases are idiopathic and the paraneoplastic form is present in 10-20% associated predominantly with hematological malignancies such as acute myelogenous leukemia, myelodysplastic syndromes and lymphoma (2,3,4)

  • drug-induced SS
    • pharmacovigilance signals have been observed with use of colony-stimulating factors, immunosuppressants, antineoplastics and antibiotics (6)

Improvement in patients with malignancy-associated Sweet's syndrome or drug-induced Sweet's syndrome may occur following successful treatment of the cancer or discontinuation of a causative medication. The therapeutic mainstay for Sweet's syndrome is systemic corticosteroids

  • dapsone has been used as either monotherapy or in combination therapy (4,5) Other agents used in the management of this condition include potassium iodide, colchicine, Indomethacin, clofazimine, cyclosporine, and antibiotics
  • corticosteroids continue to be efficacious first-line therapy for the majority of patients (7)

Reference:

Related pages:

pyoderma gangrenosum