500
A 52-year-old man is evaluated in the hospital for several skin lesions on his back, chest, and arms. The patient has acute myeloid leukemia and was hospitalized after developing neutropenic fever 10 days following his initial course of chemotherapy. The skin lesions first appeared the day his fever started. Medications are vancomycin and cefepime.
On physical examination, temperature is 38.4 °C (101.2 °F), blood pressure is 110/70 mm Hg, pulse rate is 95/min, and respiration rate is 18/min. A representative skin lesion is shown.
Hemoglobin 8.2, Leukocyte count 400/µL with 95% neutrophils, Platelet count 10,000/µL (10 × 109/L)
Metabolic profile -Normal, Urinalysis- Normal, Chest radiograph is normal. Blood cultures show no growth to date.
What is the name of the syndrome?
What is Sweet Syndrome
This patient has the typical skin findings characteristic of Sweet syndrome, also known as acute febrile neutrophilic dermatosis. The lesions are well demarcated with a sharp cut-off separating normal and inflamed skin. Because of the intense neutrophilic inflammatory infiltrate and accompanying papillary dermal edema, the lesions are often referred to as appearing “juicy.” Patients often have a leukocytosis with a predominance of neutrophils and bands; there may be an accompanying, albeit nonspecific, elevation in inflammatory markers such as erythrocyte sedimentation rate. Patients with Sweet syndrome may have idiopathic disease (common in older white women) or paraneoplastic Sweet syndrome, most commonly seen in patients with hematologic malignancies, particularly acute myeloid leukemia or myelodysplastic syndrome. The sharply demarcated, indurated red papules, plaques, or nodules almost always develop in the setting of fever and are often mistaken for infection. Patients may develop these lesions at any time during their treatment course. Sweet syndrome may also occur as a reaction to certain medications, particularly granulocyte colony-stimulating factors. The condition is extraordinarily responsive to treatment with glucocorticoids, with the fever stopping immediately and the lesions fading within 1 to 2 days.