Sweet Syndrome

November 1, 2007

A 66-year-old woman was hospitalized because of severe anemia secondary to myelodysplastic syndrome. She had had associated fatigue and throbbing pain in both legs for several days.

A 66-year-old woman was hospitalized because of severe anemia secondary to myelodysplastic syndrome. She had had associated fatigue and throbbing pain in both legs for several days.

The patient's temperature was 37.5°C (99.5°F); heart rate, 97 beats per minute; respiration rate, 20 breaths per minute; and blood pressure, 112/78 mm Hg. Pallor of the skin was noted. She had a localized, erythematous, violaceous, tender rash on the upper scapular regions. The lesions were pustular; some had blistered. The remaining physical findings were unremarkable.

The hemoglobin level was 8.8 g/dL; other laboratory test results were normal. Results of blood cultures were negative. A biopsy of the skin lesions revealed subepidermal and intradermal acute inflammatory changes with abscess formation. There was neutrophilic infiltration of the epidermis, consistent with Sweet syndrome.

About 20% to 25% of patients with Sweet syndrome have an underlying malignancy,1,2 which is commonly hemopoietic, especially myelodysplastic syndrome and acute myeloid leukemia.1,3-7 The syndrome is also associated with infection, autoimmune disease, and inflammatory bowel disease.

Cytokine dysregulation is thought to account for the clinical, pathological, and laboratory changes seen in Sweet syndrome. The eruptions usually appear on the face, neck, or upper extremities. When they occur on the lower extremities, the lesions may resemble erythema nodosum. The differential diagnosis of Sweet syndrome also includes pyoderma gangrenosum. Skin biopsy confirms the diagnosis.

This patient received a course of high-dose prednisone (40 mg/d) tapered over a 5-week period. Her condition steadily improved.

References:


REFERENCES:


1.

Cohen PR, Talpaz M, Kurzrock R. Malignancy-associated Sweet's syndrome: review of the world literature.

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2.

Cohen PR, Holder WR, Tucker SB, et al. Sweet's syndrome patients with solid tumors.

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1993;72:2723-2731.

3.

Reuss-Borst MA, Pawelec G, Saal JG, et al. Sweet's syndrome associated with myelodysplasia: possible role of cytokines in the pathogenesis of the disease.

Br J Haematol.

1993;84:356-358.

4.

Peters FP, Drent M, Verhaegh M, et al. Myelodysplasia presenting with pulmonary manifestations associated with neutrophilic dermatosis.

Ann Hematol.

1998;77:135-138.

5.

Urano Y, Miyaoka Y, Kosaka M, et al. Sweet's syndrome associated with chronic myelogenous leukemia: demonstration of leukemic cells within a skin lesion.

J Am Acad Dermatol.

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6.

Liu D, Seiter K, Mathews T, et al. Sweet's syndrome with CML cell infiltration of the skin in a patient with chronic-phase CML while taking imatinib mesylate.

Leuk Res.

2004;28(suppl 1):S61-S63.

7.

Ayirookuzhi SJ, Ma L, Ramshesh P, Mills G. Imatinib-induced sweet syndrome in a patient with chronic myeloid leukemia.

Arch Dermatol.

2005;141:368-370.