Skin Lesions and Neutropenic Fever in a 69-Year-Old Woman

Cameron Nichols, MD; Fnu Nutan, MD

Disclosures

June 26, 2020

Discussion

The patient in this case had tender erythematous plaques in association with fever and myelodysplastic syndrome. The differential diagnoses included infection and Sweet syndrome. The cultures showed no growth, so the patient was diagnosed with Sweet syndrome.

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is an uncommon disease that most commonly affects women aged 30-60 years.[1] It belongs to a group of neutrophilic dermatoses that also includes pyoderma gangrenosum, Behçet disease, and neutrophilic dermatosis of the dorsal hands.

The pathogenesis of Sweet syndrome is unknown. It is most commonly associated with underlying diseases.[1] Common conditions and triggers associated with the onset of Sweet syndrome include internal malignancies (15%-30%), preceding infection (25%), causative drugs (10%), and inflammatory bowel disease. The cancers most commonly associated with Sweet syndrome are hematologic, with acute myeloid leukemia and myelodysplastic syndrome being the most frequent.

Sweet syndrome can have a wide variety of presentations and mimic many other common skin disorders. Fever often precedes the skin lesions. The classic presentation involves deep red, sharply demarcated plaques or nodules that appear edematous or vesiculated (Figure 1). Ulcers or bullae may be present (Figure 2).

Figure 1.

Figure 2.

The plaques are usually distributed asymmetrically on the face, neck, upper trunk and extremities. Mucosal involvement is not usually seen. Pathergy (disease presenting at sites of trauma or needle sticks) is common and can be seen at intravenous insertion sites or other areas of trauma. The patient in this case had a papule at the site of a previous catheter insertion site on her chest.

The plaques often resemble cutaneous infections, such as cellulitis, atypical mycobacterium infection, and erysipelas (Figures 2 and 3). Thus, ruling these infections out prior to initiating therapy is important.

Figure 3.

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