Sweet’s SyndromeDz

Synonyms: Acute febrile neutrophilic dermatosis.

ICD9 Code: 695.89
ICD10 Code: L98.2

Definition: Sweet’s syndrome is a multisystem, febrile disorder accompanied by painful papules or plaques (with cutaneous neutrophilic infiltration), arthritis, and leukocytosis. Sweet’s syndrome may occur in association with infection, neoplasms, or other systemic inflammatory disorders.

Etiology: The cause is unknown; a hypersensitivity reaction is proposed.

Pathology: Sweet’s syndrome exhibits dense dermal infiltration with neutrophils, without vasculitic changes.

Demographics: Most patients are women between the ages of 30 and 60 years.

Cardinal Findings: Onset may follow upper respiratory infection. Systemic features include fever (>38°C in >80% of patients), malaise, conjunctivitis, episcleritis, iridocyclitis, oral ulcers, proteinuria, arthralgias, myalgias, and arthritis. Skin lesions appear as tender red or violaceous papules, plaques, or pustules over the face, neck, and arms. Lesions typically resolve in 4 to 8 weeks but may recur. Self-limiting, asymmetric, oligo- or polyarthritis occurs in as many as 25% of patients. Arthritis tends to parallel skin lesions and usually affects the hands, wrists, ankles, or knees. Pulmonary features (cough, dyspnea, or pulmonary infiltrates) occur in <10% of patients.

Complications: Some (15% to 25%) develop a malignancy, particularly acute myelocytic leukemia, multiple myeloma, myelodysplasia, lymphoma, or solid tumors (e.g., prostate, ovarian, testicular, and breast cancer). Nearly 50% have other underlying conditions such as inflammatory bowel disease, pregnancy, or other connective tissue diseases (RA, SLE, relapsing polychondritis, sarcoidosis, Behçet’s or Sjögren’s syndrome).

Diagnostic Tests: Laboratory testing abnormalities include anemia and elevated ESR, WBC counts, and alkaline phosphatase. Proteinuria is seen in 15%. Positive P-ANCA test results have been reported.

Diagnostic Criteria: Major criteria are (a) abrupt-onset painful plaques or nodules and (b) neutrophilic infiltrates in the dermis without leukocytoclastic vasculitis. Minor criteria are (a) preceded by fever or infection; (b) accompanied by fever, arthralgia, conjunctivitis, or underlying malignant lesion; (c) leukocytosis; (d) good response to steroids but not antibiotics; and (e) increased ESR. Diagnosis requires that both major and two minor criteria be fulfilled.

Differential Diagnosis: Erythema elevatum diutinum, erythema nodosum, pyoderma gangrenosum, SLE, and Still’s disease should be considered.

Therapy: Oral steroids (e.g., prednisone 40–60 mg/day) are effective. Steroids should be tapered over 4 to 6 weeks. Other therapies, including aspirin, NSAIDs, dapsone, colchicine, sulfapyridine, and potassium iodide, have had some success.

Comment: Onset of Sweet’s syndrome underscores the need for a thorough history and physical examination to exclude an associated malignancy.

BIBLIOGRAPHY

Cohen PR, Kurzrock R. Sweet’s syndrome: a review of current treatment options. Am J Clin Dermatol 2002;3:117–131.PMID:11893223

Fett DL, Gibson LE, Su WPD. Sweets syndrome: systemic signs and symptoms. Mayo Clin Proc 1995;70:234–240.PMID:7861810