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Sweet Syndrome

Acute febrile neutrophilic dermatosis: rapid-onset painful plaques with fever

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Sweet Syndrome?

Sweet syndrome was first described by Robert Douglas Sweet in 1964. It is classified into three subtypes: classical (most common, 70%, F:M 4:1, age 30-50), malignancy-associated (15-20%, often AML, F:M ~1:1), and drug-induced (G-CSF, all-trans retinoic acid, hydralazine, sulfa drugs, minocycline, OCPs).

Pathogenesis: hypersensitivity reaction with cytokine dysregulation (IL-1, IL-6, IL-8, GM-CSF, IFN-γ), neutrophil chemotaxis without primary vasculitis. Histopathology: dense dermal neutrophilic infiltrate with leukocytoclasia, papillary dermal edema, no vasculitis (early), no infection.

Diagnosis (Su criteria): two major criteria (abrupt painful erythematous plaques + dense neutrophilic infiltrate without vasculitis) + two minor criteria (fever >38°C, association with infection/inflammation/malignancy/drug/pregnancy, response to corticosteroids/KI, abnormal labs - 3/4: ESR>20, CRP+, WBC>8000, neutrophils>70%). Treatment: systemic corticosteroids first-line (rapid response within 1-3 days), potassium iodide, colchicine, dapsone for refractory.

Symptoms

Sudden onset of painful, tender erythematous plaques and nodules
Lesions appear pseudovesicular (mamillated, juicy appearance)
Distribution: face, neck, upper extremities, dorsa of hands, trunk
Fever >38°C (preceding or concomitant)
Malaise, fatigue, arthralgia, myalgia
Conjunctivitis, episcleritis, oral aphthous ulcers
Pulmonary infiltrates, hepatic, renal, neurologic involvement (extracutaneous)
Pathergy phenomenon (lesions at trauma sites - 30%)

Risk Factors

Recent upper respiratory or GI infection (Yersinia, Streptococcus)
Inflammatory bowel disease (Crohn, UC)
Hematologic malignancy (AML most common, 15% of malignancy-associated)
Solid tumors (GI, GU, breast)
Pregnancy (rare classical subtype)
Drugs: G-CSF, all-trans retinoic acid (ATRA), trimethoprim-sulfamethoxazole, minocycline, OCP, hydralazine, NSAIDs
Female gender (4:1 in classical), age 30-50
Connective tissue disease, behçet

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Sudden painful red-purple plaques with fever
  • Rash with fever in patient with leukemia/lymphoma
  • Painful skin lesions after starting new medication (especially G-CSF)
  • Recurrent fever and skin rash with arthralgia
  • Skin lesions in pregnant patient with fever
  • Plaques developing at trauma sites (pathergy)
  • Failure to respond to antibiotics for presumed cellulitis

Treatment Methods

01
Systemic corticosteroids (prednisone 0.5-1 mg/kg/day) - first-line, dramatic response in 1-3 days
02
Potassium iodide (300-900 mg/day) - alternative first-line
03
Colchicine (0.5 mg BID-TID) - effective option
04
Dapsone (50-150 mg/day) - steroid-sparing
05
Indomethacin (50 mg TID) - alternative
06
Cyclosporine, methotrexate, anti-TNF agents (refractory)
07
Anakinra (IL-1 antagonist - refractory cases)
08
Treat underlying trigger: antibiotics for infection, IBD therapy, malignancy treatment
09
Discontinue offending drug (drug-induced)
10
Topical corticosteroids (limited cutaneous disease)
11
Workup for malignancy: CBC with differential, blood smear, age-appropriate cancer screening
12
Recurrence common (~30%) - lifelong follow-up
13
Slow corticosteroid taper over 4-6 weeks to prevent recurrence

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.