Corticosteroid Resistant Sweet Syndrome in the Setting of Ac
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A 52 year old man with a past medical history remarkable for Type 2 Diabetes presented with a 1-week history of a painful, pruritic facial rash that subsequently spread to the trunk and bilateral upper and lower extremities. The rash continued to
progress despite four days of methylprednisolone (4 mg) and cephalexin (500 mg), prescribed by his primary care physician for presumed acute folliculitis prior to dermatology referral.

Review of systems was remarkable for malaise, fatigue, dizziness, headache, epistaxis, easy bruising and a 10 pound unintentional weight loss in the past 2 months. Examination was significant for pink, deep red, and purple edematous nodules and plaques on the scalp, face, trunk, and bilateral upper and lower extremities. Punch biopsies for histopathology and tissue cultures were obtained. The patient was started on daily oral prednisone (80 mg) and BD triamcinolone ointment (0.1%) for suspected SS, while awaiting the laboratory, microbiology, and histopathology results.

Histopathology from two punch biopsies of lesions on the left upper arm and shoulder showed papillary dermal edema, with a diffuse neutrophilic infiltrate in the reticular dermis, consistent with SS. His initial CBC revealed pancytopenia; a manual differential revealed 9% blasts in the peripheral blood. He was referred urgently to oncology for suspected myeloid malignancy, and subsequent bone marrow biopsy confirmed a diagnosis of AML. Cytogenetic analysis revealed a 5q deletion and monosomy 7.

The patient was admitted to the hospital for induction chemotherapy. Upon admission, it was noted that his rash continued to progress despite 8 total days of oral corticosteroid therapy. Given the lack of response to treatment, he was started on daily colchicine (0.6mg) and transitioned to daily IV methylprednisolone (90 mg) during his hospitalization. His colchicine was increased to 1.2mg daily dose for two weeks and he was discharged home on a prednisone taper (reduction by 20 mg every 3-5 days).

Clinical improvement was noticed on day 7 of chemotherapy and week 3 of systemic corticosteroids. His SS showed significant improvement after 32 days of chemotherapy with liposomal daunorubicin and cytarabine (44 mg/m2) and 6 weeks of systemic corticosteroids.

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