A Collection of Cases of Sweet Syndrome
Sweet Syndrome Associated With Acute Myeloid Leukemia
Hendy B. Jean, MD; Htet Htet Win, MD; Sabita Sarkar, MD; Minale Desta, MD; Ashhar Ahmed, MS-III; and Harish Patel, MD
Kingsbrook Jewish Medical Center, Brooklyn, New York
Jean HB, Win HH, Sarkar S, Desta M, Ahmed A, Patel H. Sweet syndrome associated with acute myeloid leukemia. Consultant. 2017;57(5):300-301.
A 56-year-old man presented to our emergency department (ED) with fever, chills, dyspnea, diarrhea, and vomiting. The patient had been in his usual state of normal health until 2 weeks prior, when he had visited a different ED for a lump in his left buttock. The lump was described as a red-to-purple nodule that was nonfluctuant and without crepitation. He had been discharged home on a regimen of oral ciprofloxacin and metronidazole.
One week thereafter, the lesion had not improved, and the patient presented to another health care facility where incision was performed without drainage of pus.
History and physical examination. On presentation to our ED, the patient reported having fever, chills, dyspnea, diarrhea, and vomiting but denied cough, sputum production, sick contacts, or recent travel. On physical examination, he appeared lethargic and was febrile (temperature, 38.9°C), with a blood pressure of 152/92 mm Hg and a heart rate of 102 beats/min.
Skin examination showed a red-to-purple lesion in the left buttock (Figure 1) without fluctuation or crepitation, as well as 3 new lesions on the left forehead (Figure 2), the left tragus (Figure 3), and the right side of the upper lip. Cardiovascular examination findings were remarkable for tachycardia and an irregular pulse. The rest of the physical examination findings, including examination of the lungs, abdomen, rectum, and extremities, were unrevealing.
Diagnostic tests. Results of initial laboratory studies were notable for a white blood cell count of 29,700/µL, a blood urea nitrogen level of 52 mg/dL, and a creatine level of 1.5 mg/dL. Electrocardiography showed atrial fibrillation with rapid ventricular response. Chest radiography revealed no acute chest pathology. Computed tomography scanning of the abdomen revealed no acute intra-abdominal process, abscess, or collection but did show soft tissue swelling of the left buttock without abscess or collection.
The patient initially was treated for presumed sepsis with broad-spectrum antibiotics. Due to the severity and complexity of the disease on presentation, clinicians from multiple specialties (including cardiology, infectious disease, gastroenterology, and general surgery) were involved in the management of the patient’s condition. Pan-culture test results remained negative for pathogens throughout his entire hospital stay, as did the results of stool DNA tests for Clostridium difficile.
Another incision performed at the site of the left buttock lesion revealed no abscess or pus collection. His diarrhea had been persistent and unresponsive to antibiotics and antidiarrheal therapy. Findings of a colonoscopy performed by the gastroenterologist ruled out inflammatory bowel disease. Despite treatment, the leukocytosis, skin lesions, and general condition of the patient worsened, prompting consultation with specialists in hematology-oncology and dermatology.
Diagnosis. Based on the results of bone marrow biopsy, flow cytometry testing, and skin biopsy of the lesions on his left forehead, left tragus, and left buttock, the patient received a diagnosis of acute myeloid leukemia (AML) associated with Sweet syndrome.
Discussion. Sweet syndrome, also called acute febrile neutrophilic dermatosis, is an uncommon inflammatory disorder characterized by the abrupt onset of painful dermatologic lesions. Its presentation ranges from classic Sweet disease, which can occur after mild respiratory illness, to a more aggressive neutrophilic process, which may be associated with other inflammatory diseases or malignancies, especially hematologic malignancies.
The initial presentation of the patient pointed more toward an infectious process; however, as data from the history, physical examination, laboratory studies, imaging studies, and his response to therapy during the hospital course were collected, the clinical picture increasingly pointed toward a more intricate and complicated disease process.
Among the differential diagnoses is pyoderma gangrenosum, which can present in a similar fashion to Sweet syndrome with constitutional symptoms, gastrointestinal tract symptoms in association inflammatory bowel disease, and skin lesions. The skin biopsy report in our patient’s case ruled out pyoderma gangrenosum, however.
Outcome of the case. Once the diagnosis of Sweet syndrome in association with AML had been established, the patient was started on prednisone therapy, which led to dramatic improvement in the skin lesions. Further therapy, including chemotherapy, eventually resulted in resolution of all the man’s physical symptoms as well as the neutrophilia. He was discharged in stable condition after being transferred to another facility for chemotherapy, and a plan for follow-up was made with his oncologist.