A Collection of Cases of Sweet Syndrome
Sweet Syndrome Associated With Cocaine Use
Samuel Slone, BS, and Igor Sunjic, MD
University of South Florida Morsani College of Medicine, Tampa, Florida
Slone S, Sunjic I. Sweet syndrome associated with cocaine use. Consultant. 2017;57(5):301-303.
A 37-year-old woman with a history of intravenous hydromorphone use and crack cocaine use presented to the emergency department with a 1-day history of a pustular rash associated with burning pain.
History. The pustular lesions had begun on her hands bilaterally and had quickly progressed over her upper extremities, chest, back, abdomen, lower extremities, and face over the course of 24 hours, without mucosal involvement. She denied fever, joint pain, chest pain, abdominal pain, dyspnea, or recent illnesses. She stated that she had taken a 4-day course of amoxicillin prior to admission for a self-diagnosed ankle infection. She had received the antibiotics from a friend. She reported having had a similar rash 1 year prior to presentation that had been localized to her posterior calves and had occurred after the use of clindamycin for an unknown infection.
She initially presented to an outside hospital and had been given vancomycin, piperacillin-tazobactam, and acyclovir out of concern for widespread infection. She had been transferred to our facility for further evaluation.
Physical examination. The patient was an uncomfortable-appearing woman with a diffuse, widespread pustular rash. She was febrile (temperature, 39.4°C) and had a heart rate of 120 beats/min. The rash involved the bilateral hands, upper and lower extremities, and feet (Figures 1-3), as well as the face, neck, and superior chest. There was minimal involvement of the posterior lower extremities and posterior trunk. Scattered pustules were present on the abdomen and labia. Vesicular lesions were present on the right volar distal forearm, and larger ulcerated lesions were present on the dorsal hands and forearms (Figure 4), with a positive Nikolsky sign. There were no lesions in the oral mucosa or on the palms or soles.
Diagnostic tests. The initial white blood cell (WBC) count was 8570/µL with a neutrophilic predominance (72%). The C-reactive protein (CRP) level was 25.21 mg/L, and the erythrocyte sedimentation rate (ESR) was 53 mm/h. Blood, fungal, wound, anaerobic, and acid-fast cultures were taken, and all were without growth throughout admission. Results of polymerase chain reaction tests for varicella-zoster virus and herpes simplex virus were negative. The results of all other initial laboratory tests, including a complete blood cell count (CBC) and reticulocyte count, were unremarkable.
Results of a skin biopsy of the left thigh showed a wedge-shaped lesion with partial epidermal necrosis, blister formation, and diffuse neutrophilic infiltrate, with nuclear fragmentation in the upper portion of the reticular dermis (Figures 5 and 6). The histologic findings had the combined features of Sweet syndrome, also known as acute febrile neutrophilic dermatosis.
Discussion. Sweet syndrome is an uncommon inflammatory disorder characterized by the abrupt appearance of burning, painful, erythematous papules, plaques, and cutaneous nodules that are typically accompanied by fever and leukocytosis with neutrophil predominance.1 Sweet syndrome is typically subdivided into 3 categories: classic, malignancy-associated, and drug-induced.
While most cases of classic Sweet syndrome are idiopathic, cases have been linked to infections (particularly in the upper respiratory and gastrointestinal tracts), inflammatory bowel disease, and pregnancy.1 There have also been less-documented associations with autoimmune conditions, primary immunodeficiency, and other infections (eg, human immunodeficiency virus, tuberculosis).
Malignancy-associated Sweet syndrome accounts for approximately 21% of cases.2 A stronger association exists with hematologic malignancies than with solid tumor cancers.3 Acute myeloid leukemia has the strongest association with Sweet syndrome, accounting for approximately 42% of associated hematologic malignancies.4
Drug-induced Sweet syndrome has been linked to the use of a variety of illicit drugs and medications. Notably, it has been linked to the use of cocaine. In terms of medications, it is most strongly associated with granulocyte-colony stimulating factor,5 and the condition has also been linked to antibiotics, nonsteroidal anti-inflammatory drugs, and antineoplastic agents.
In order to receive a diagnosis of Sweet syndrome, a patient must exhibit the 2 major criteria: abrupt onset of painful, erythematous plaques or nodules, and histopathologic evidence of a dense, neutrophilic infiltrate without evidence of leukocytoclastic vasculitis.6 The patient must also exhibit 2 of 4 minor criteria: pyrexia above 38°C; association with an underlying hematologic or visceral malignancy, inflammatory disease, or pregnancy, or preceded by an upper respiratory tract or gastrointestinal tract infection or vaccination; excellent response to treatment with systemic corticosteroids or potassium iodide; and abnormal laboratory values at presentation (3 of the following 4: ESR greater than 20 mm/h, elevated CRP; a WBC count above 8000/µL, or a differential cell count showing greater than 70% neutrophils).6 Our patient met these diagnostic criteria and, coupled with the overall clinical picture, a diagnosis of Sweet syndrome was confirmed.
Outcome of the case. The patient was placed on 60 mg of oral prednisone daily, with a planned 6-week taper. Over the first 48 hours after medication administration, the patient’s symptoms demonstrated significant improvement. She continued to improve on the corticosteroid taper.
It was believed that the inciting event for the development of Sweet syndrome was either idiopathic or was related to the patient’s use of cocaine. Given that her initial CBC and reticulocyte count findings were unremarkable, further workup for acute myeloid leukemia was not pursued.
- Cohen PR. Sweet’s syndrome—a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007;2:34.
- Cohen PR, Kurzrock R. Sweet’s syndrome and cancer. Clin Dermatol. 1993;11(1):149-157.
- Raza S, Kirkland RS, Patel AA, Shortridge JR, Freter C. Insight into Sweet’s syndrome and associated-malignancy: a review of the current literature. Int J Oncol. 2013;42(5):1516-1522.
- Cohen PR, Talpaz M, Kurzrock R. Malignancy-associated Sweet’s syndrome: review of the world literature. J Clin Oncol. 1988;6(12):1887-1897.
- White JML, Mufti GJ, Salisbury JR, Du Vivier AWP. Cutaneous manifestations of granulocyte colony-stimulating factor. Clin Exp Dermatol. 2006;31(2):206-207.
- von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol. 1994;31(4):535-556.