Sweet's Syndrome

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Text of Sweet's Syndrome

  • 1. Sweets Syndrome Allison Dupont AM Report 1/17/06
  • 2. Definition
    • Sweets syndrome (acute febrile neutrophilic dermatosis) is characterized by:
      • Fever
      • Peripheral neutrophilia
      • Painful red skin papules, nodules and/or plaques
      • Neutrophilic infiltration of skin (particularly the dermis)
  • 3. Clinical Presentation
    • Fever (>38 C) can be intermittent and may precede skin manifestations by days to weeks.
    • Systemic symptoms may include headache, myalgia, arthralgia, and general malaise.
    • Cutaneous lesions consist of erythematous to violaceous tender papules which may coalesce to form plaques.
      • The plaques are not pruritic.
      • Most often found on the face, neck and upper extremities (especially the dorsum of the hands), but can occur anywhere.
      • Lesions on the lower extremities may resemble erythema nodosum.
  • 4.
  • 5.
  • 6. Clinical Presentation
    • Oral ulcers (more common in patients with Sweets syndrome and a hematologic malignancy.
    • Ocular involvement (uncommon in malignancy-associated and drug-induced Sweets syndrome).
      • Conjunctivitis, episcleritis
  • 7. Clinical Presentation
    • Involvement of internal organs may occur leading to:
      • Alveolitis
      • Sterile osteomyelitis
      • Involvement of liver, pancreas, and/or kidneys
      • Neurologic and psychiatric changes
  • 8. Laboratory Findings
    • Lab findings are nonspecific.
    • Majority will have peripheral neutrophilia.
    • Other possible lab abnormalities include:
      • Elevated sedimentation rate
      • Elevated C-reactive protein
      • Leukocytosis
    • Consider evaluation of hepatic and renal function.
  • 9. Pathology
    • Sweets syndrome characteristically involves dense neutrophilic infiltration of the dermis +/- dermal edema.
    • Neutrophil karyorrhexis is commonly seen.
    • There is no involvement of the vasculature of the skin and no necrosis (in contast to pyoderma gangrenosum).
  • 10.
  • 11. Diagnosis
      • Major Criteria
    • Abrupt onset of painful erythematous plaques or nodules.
    • Histopathologic evidence of a dense dermal neutrophilic infiltrate without vasculitis .
    • Minor Criteria
    • Fever (>38 C)
    • Association with an underlying hematological/visceral malignancy, inflammatory disease, or pregnancy or preceded by an upper respiratory or GI infection or vaccination.
    • Excellent response to treatment with systemic corticosteroids.
    • Peripheral neutrophilia (>70% neutrophils)
  • 12. Associated Conditions
    • Sweets syndrome is associated with an underlying disease or condition in up to 50% of patients.
    • Sweets syndrome may be the presenting sign and the underlying disease may not become apparent for several years after Sweets syndrome occurs.
    • There is a female predominance except in the case of malignancy-associated Sweets syndrome.
  • 13. Associated Conditions
    • Malignancy
        • Approximately 21% of patients with Sweets syndrome have a malignancy.
        • 15% hematological (most commonly AML)
        • 6% solid tumors (most commonly carcinomas of the GU tract, GI tract, or breast)
    • Infections
        • Mostly of the upper respiratory or GI tract
        • Streptococcus, mycobacterium, Yersinia, Salmonella, Shigella
  • 14. Associated Conditions
    • Inflammatory bowel disease
        • Sweets syndrome may occur alone or in combination with pyoderma gangrenosum.
    • Pregnancy
    • Other conditions with a possible association:
        • -Sarcoidosis
        • -Rheumatoid arthritis
        • -Thyroid disease (Graves disease and Hashimotos thyroiditis)
  • 15. Drug-induced Sweets syndrome
    • Criteria slightly different than classical syndrome.
    • Temporal relationship between drug ingestion/injection and clinical presentation.
    • Resolution of lesions/symptoms after withdrawal of drug or treatment with corticosteroids.
  • 16. Drug-induced Sweets syndrome
    • G-CSF is responsible for the majority of cases.
    • Other possible causes: furosemide, lithium, hydralazine, trimethoprim-sulfamethoxazole, and oral contraceptives.
  • 17. Pathogenesis
    • Etiology of Sweets syndrome is unknown.
    • Presumed to be due to a hypersensitivity reaction to an eliciting antigen which leads to stimulation of cytokine release.
    • Cytokines precipitate neutrophil activation and infiltration.
    • Response to treatment with corticosteroids supports this etiology.
  • 18. Pathogenesis
    • The source of the eliciting antigen may be diverse, including bacterial, viral or tumoral antigens.
  • 19. Treatment
    • Gold standard: Systemic corticosteroids
      • Start at 1 mg/kg/day prednisone with long taper (4-6 weeks) to 10 mg/day.
      • Many patients require several months of
      • 10-30 mg/day to suppress recurrences.
    • Localized Sweets syndrome can sometimes be treated with high-potency topical corticosteroids .
  • 20. Treatment
    • Other first-line agents include:
    • Oral potassium iodide
        • -Systemic symptoms resolve in 1-2 days.
        • -Dermatitis resolves in 3-5 days.
    • Colchicine
    • -Systemic symptoms resolve in 2-3 days.
    • -Dermatitis resolves in 2-5 days.
  • 21. Alternative therapies
    • Indomethacin
    • Clofazimine
    • Cyclosporine
    • Dapsone
  • 22. References
    • Burall, Barbara M.D. Sweets syndrome (acute febrile neutrophilic dermatosis). Dermatology Online Journal 5(1):8.
    • Cohen, Philip R. MD, Kurzrock, Razelle MD. Sweets syndrome revisited: a review of disease concepts. International Journal of Dermatology . Volume 42, Issue 10. October 2003.
    • Cohen, Philip R. Sweets syndrome. Orphanet . October
    • 2003.
    • Federman et al. Cutaneous manifestations of malignancy. Postgraduate Medicine Online . January 2005.
    • Joe, Edwin K. MD. Sweets syndrome. Dermatology Online Journal 9(4):28.
    • Moschella, Samuel L. MD. Neutrophilic dermatoses. UpToDate .
    • Sweets Syndrome. Dermis.net .