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Sweet syndrome


Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports

Sweet syndrome is a neutrophilic dermatosis that clinically presents with the sudden onset of tender, well-demarcated, edematous, erythematous plaques or nodules on the face, neck, upper trunk, and/or extremities. A pseudovesicular or pustular morphology is possible. Patients can also develop fever, leukocytosis, arthralgias, myalgia, general malaise, and, infrequently, internal organ involvement. Histopathology shows a dense neutrophilic infiltrate in the upper dermis without evidence of primary leukocytoclastic vasculitis. Neutrophilic dermatosis of the dorsal hands is an anatomically limited subset of Sweet syndrome.

Management Strategy

Sweet syndrome is often idiopathic, but may be malignancy-associated (most commonly acute myelogenous leukemia, but also lymphomas, dysproteinemias, and carcinomas), drug-induced, or related to conditions such as inflammatory bowel disease, infection (commonly Streptococcus or Yersinia ), radiation, and pregnancy. Workup includes skin biopsy, complete physical examination, and laboratory studies. Treatment of the underlying condition or discontinuation of the causative medication may lead to resolution of symptoms. The standard treatment is corticosteroids. Second- and third-line therapies may be effective for patients with contraindications to corticosteroids or recalcitrant disease. Necrotizing Sweet syndrome should be distinguished from the clinically-resembling necrotizing fasciitis, which has a drastically distinct approach to treatment.

Specific Investigations

  • Complete blood count with differential

  • Erythrocyte sedimentation rate or C-reactive protein

  • Cultures as indicated by history and physical examination

  • Malignancy screening: age-appropriate screening, evaluation if hematologic abnormalities exist, and consideration of serum protein/serum immunofixation electrophoresis; bone marrow biopsy may be considered

  • Pregnancy test (in women of childbearing potential)

  • Medication history

  • Skin biopsy

First-Line Therapies

  • Oral corticosteroids

  • C

  • Topical and intralesional corticosteroids

  • C

Sweet’s syndrome: a review of current treatment options

Cohen PR, Kurzrock R. Am J Clin Dermatol 2002; 3: 117–31.

Corticosteroids (oral prednisone 0.5–1.5 mg/kg daily tapered over 4–6 weeks) results in rapid relief of systemic symptoms (1–2 days) and skin lesions (2–10 days). Relapse may occur.

High-potency topical and intralesional corticosteroids may be useful as monotherapy or adjuvant treatment for limited or mild disease.

Second-Line Therapies

  • Potassium iodide

  • C

  • Colchicine

  • C

  • Indomethacin

  • C

  • Pulse intravenous corticosteroids

  • D

  • Dapsone

  • D

  • Clofazimine

  • D

  • Doxycycline

  • E

  • Metronidazole

  • E

Potassium iodide in refractory, recurrent pediatric Sweet syndrome: guidance in dosing and monitoring

Tangtatco JA, Ho N, Drucker A, et al. Pediatr Dermatol 2018; 35: 271–3.

Supersaturated solution of potassium iodide (SSKI) dosing starts at three drops three times daily, is increased by one drop per dose up to 10 drops (500 mg) three times daily until clear, then tapered. SSKI, which is contraindicated in pregnancy, hyperkalemia, and renal disease, requires monitoring of laboratory values including electrolytes and renal and thyroid function tests.

Potassium iodide tablets (300 mg) are usually administered three times daily. Dilution of the liquid formulation or the capsule formulation may help reduce the bitter taste.

Long-term suppression of chronic Sweet’s syndrome with colchicine

Ritter S, George R, Serwatka LM, et al. J Am Acad Dermatol 2002; 47: 323–4.

Successful treatment using 0.6 mg colchicine twice daily.

Colchicine 0.5 mg orally three times daily over 10–21 days was effective in 18 patients. Patients may experience gastrointestinal symptoms such as nausea and diarrhea that can improve with tapering.

Neutrophilic dermatosis of the dorsal hands treated with indomethacin

Kaur MR, Bazza MA, Ryatt KS. Br J Dermatol 2006; 154: 1089–90.

A patient treated with indomethacin 150 mg/day for 1 week followed by 100 mg/day for 2 weeks.

This dosing was effective in another study involving 18 patients. Seventeen patients responded within 48 hours without relapse.

Association of acute neutrophilic dermatosis and myelodysplastic syndrome with (6;9) chromosome translocation

Megarbane B, Bodemer C, Valensi F, et al. Br J Dermatol 2000; 143: 1322–4.

Pulse corticosteroid therapy successfully used for Sweet syndrome.

Intravenous methylprednisolone up to 1000 mg/day can be used for 3–5 days, followed by a taper of oral corticosteroids with or without another immunosuppressant.

Treatment of giant cellulitis-like Sweet syndrome with dapsone

Koketsu H, Ricotti C, Kerdel FA. JAMA Dermatol 2014; 150: 457–9.

Neutrophilic dermatosis of the dorsal hands: response to dapsone monotherapy

Ramos FS, Ferrera FR, Rabay FMO, et al. An Bras Dermatol 2018; 93: 730–2.

Two reports describing excellent response to dapsone 100 mg/day.

Alternatively, sulfapyridine 1–4 g/day can be used.

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