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Introduction: Sweet's syndrome (ss), or acute febrile neutrophilic dermatosis, is characterized by sudden onset of fever, leukocytosis and erythematous plaques or nodules infiltrated by neutrophils. There are three main clinical settings in which Sweet's syndrome has been described: The diagnosis of Sweet’s syndrome often has a temporal association with the discovery or relapse of cancer as reported in this case.
Case Report: A 64-year-old woman with recurrent breast cancer on targeted and hormonal therapy presented to the ED with acute onset of fever and diffuse non-tender body rash. Vitals suggested sepsis and she was treated with broad spectrum antibiotics. A chest xray, viral panel, blood cultures, echocardiogram and urinalysis were unrevealing. Erythrocyte sedimentation rate and C-reactive protein were elevated. There were 90% neutrophils. When her erythematous plaques became vesicular, she was treated with IV Acyclovir for possible disseminated zoster. This was discontinued when biopsy for HSV/HZV returned negative. Skin biopsy, however, showed dense neutrophilic infiltration consistent with Sweet Syndrome. Treatment with prednisone resulted in rapid clinical improvement.
Discussion: Historically, the diagnosis of SS requires the presence of painful erythematous lesions (both Major Criteria and two of four Minor Criteria). The absence of pain and tenderness in this patient made the diagnosis challenging. After extensive evaluation to rule out infectious etiology, it was revealed through skin biopsy that her syndrome was more consistent with Sweet Syndrome despite having non-tender lesions. Given her recent recurrence of breast cancer, it was thought that her SS was more consistent with Malignancy-Associated SS (MASS) subtype. MASS is most commonly associated with hematological malignancies such as AML, but also occurs coincident with solid tumors, such as carcinomas of GU organs, breast, and GI tract. In patients with a previous history of cancer, the diagnosis of SS usually heralds the onset of recurrence. Sweet syndrome may precede, follow, or appear concurrently with a malignancy. This patient was found to have a recurrence of breast cancer in the form of bone metastases two months prior to admission. In summary, this case demonstrates an atypical painless presentation of SS and how evaluation for malignancy is indicated for patients with SS, particularly when there is an absence of other explanations such as recent infection, inflammatory disease or drug exposure. Treatment : Prednisone 0.5mg to 1mg/kgper day. Symptoms usually improve within 48 hours and skin lesions resolves within 1 to 2 weeks. Steroid is then taper over course of 4 to 6 weeks.
Graduate Medical Education
Conference / Event Name
Academic Achievement Day, 2020
Providence St. Vincent, Internal Medicine Residency, Portland, Oregon
Nguyen, Khoi and Schafir, Alex, "Unpainfully Sweet" (2020). Providence St. Vincent Internal Medicine. 1.