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Chapter 21: Sweet Syndrome

Razelle Kurzrock; Jerome T. Cohen; Philip R. Cohen

SWEET SYNDROME

Therapeutic Approach

Sweet syndrome (acute febrile neutrophilic dermatosis) is usually characterized by the sudden onset of pyrexia, neutrophilia in the blood and upper dermis, and tender erythematous skin lesions. In the author’s experience, topical therapy is usually initiated with a high-potency corticosteroid cream or ointment; first-line systemic therapies include corticosteroids, colchicine, and potassium iodide whereas second-line therapies—usually reserved for recalcitrant cases—include indomethacin, dapsone, clofazimine, and cyclosporin. See Table 21-1.

Table 21-1Sweet Syndrome Treatment Table
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Table 21-1 Sweet Syndrome Treatment Table
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MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTSa SUGGESTED MONITORINGb LEVEL OF EVIDENCEc (REFERENCE)
Topical Therapy
Corticosteroids CS, DS, MS Anti-inflammatory Grp 1 (HP) crm or oint BID-TID Skin atrophy None III1-3
Systemic Therapyd,e
Acitretin CS Retinoid; inhibits neutrophil migration 10-50 mg/d Bone, hematologic, hypothyroidism, lipid, mucocutaneous, neurologic, ocular, teratogenicity CBC, serum chemistries, lipid (cholesterol and triglycerides), pregnancy test, and pregnancy prevention III4
Adalimumab (SC) CS Inhibits TNF-α 40 mg QO wk Congestive heart failure, hematologic, injection site reaction, liver (function test abnormality or hepatitis), lymphoma, neurologic, opportunistic infection, tuberculosis CBC, serum chemistries, hepatitis B serologies, hepatitis C serologies, HIV, purified protein derivative, and/or quantiferon-tuberculosis gold III5
Anakinra (SC) CS ILK-1 receptor antagonist 100 mg/d Allergic reaction, anaphylactic reaction, gastrointestinal toxicity, headache, infection, injection site reaction, neutropenia CBC, serum chemistries, purified protein derivative, and/or quantiferon-tuberculosis gold III5,6
Clofazimine CS, MS Neutrophil fxn altered and mobility inhibited 100 mg QOD to 200 mg QD Acquired ichthyosis, gastrointestinal toxicity, orange-brown skin color Serum chemistries III7,8
Colchicine CS, MS Decreased neutrophil chemo, adhe, and degran 0.5 mg QD-TID Gastrointestinal toxicity, headache CBC, serum chemistries, urinalysis IIB9
Cyclophosphamide CS, MS Alkylating agent; dep B-cell > T-cell fxn 50 mg/d Cancer (leukemia, lymphoma, squamous cell carcinoma, and urinary bladder transitional cell carcinoma), hematologic, reproductive (amenorrhea and azoospermia), urinary bladder CBC III10
Cyclosporine CS, MS Inhibits neutrophil chemo and migration 2-10 mg/kg/d Gastrointestinal toxicity, headache, hypertension, neurologic, renal; decrease: magnesium; increase: potassium, uric acid, lipid Blood pressure, CBC, serum chemistries, lipid (cholesterol and triglycerides), magnesium, uric acid III11
Dapsonef CS, MS Inhibit neutrophil myelo and chemo 50-200 mg/d gastrointestinal toxicity, hematologic, hemolytic anemia, liver (function test abnormality or hepatitis), methemoglobinemia, peripheral neuropathy, psychosis, DRESS syndrome CBC, serum chemistries, G6PD, urinalysis III1,12,13
Etanercept CS Inhibits TNF-α 1 mg/kg or 25-50 mg TW – Q wk Congestive heart failure, hematologic, injection site reaction, liver (function test abnormality or hepatitis), lymphoma, neurologic, opportunistic infection, tuberculosis CBC, serum chemistries, hepatitis B serologies, hepatitis C serologies, HIV, purified protein derivative, and/or quantiferon-tuberculosis gold III5
Immunoglobulin (IV) CS, DS, MS Antibody production suppression 0.5-1 g/kg/d × 1-2 d; +/–q3wk Anaphylactic reaction, fluid overload, hematologic, infection, neurologic, thromboembolic event CBC, serum chemistries, hepatitis B serologies, hepatitis C serologies, HIV, immunoglobulin A

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