AZACITIDINE-INDUCED SWEET SYNDROME: A UNIQUE PRESENTATION

Abstract

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Sweet's Syndrome (SS) or febrile neutrophilic dermatosis, was first described in 1964 by Robert Sweet and has a female predilection with a female to male ratio of 4:1, with the typical age of onset between 30 and 60 years of age [1]. SS can either be idiopathic, pregnancy, or drug-induced, associated with infections or with malignancies [2]. We describe a unique presentation of SS in an elderly male with myelodysplastic syndrome (MDS) receiving Azacytidine (AZA). CASE PRESENTATION: A 76-year-old Caucasian male with medical history pertinent for MDS reported a new rash and fever which started immediately after completing his second 7-day cycle of AZA. Vitals were significant for hypotension and tachycardia. Physical exam was remarkable for nodules and erythematous plaques diffusely spread across the trunk, back, upper and, lower extremities. CBC showed pancytopenia and elevated inflammatory markers. The patient was adequately resuscitated, however, required low dose pressors, transfused two units of packed red blood cells, and started on broad-spectrum antibiotics. He was admitted for sepsis and neutropenic fever. Histopathology from a punch biopsy of the right calf plaque showed mild spongiosis, acanthosis, and papillary dermal edema. The dermis contained patchy perivascular inflammation, which was lymphoplasmacytic and neutrophilic. Stains for infectious agents were negative. Tissue cultures were negative for any bacterial or fungal growth. A diagnosis of SS, secondary to AZA was made. The patient was started on systemic steroids with a slow taper and made a full recovery ten days after. DISCUSSION: All five diagnostic criteria for drug-induced Sweet's syndrome proposed by Walker and Cohen were met [3]. Including an abrupt onset of painful erythematous plaques or nodules, histopathological evidence, temperature > 38°C, a temporal relationship between administration of AZA and development of the syndrome, and the resolution of symptoms when corticosteroids were given and AZA was withheld [4]. AZA is an antineoplastic agent used in the treatment of MDS. Recently, there has been an uptick in reports on AZA-associated SS [5]. The average time to resolution of clinical symptoms in drug-induced SS is reported to be around 8 days, consistent with the patient presentation [6]. CONCLUSIONS: Our case is unique in that the patient developed diffuse lesions, differing from current literature, which supports skin manifestations at the sites of injections. Prognosis is poor in patients with MDS who have SS [7]. Hence, a timely diagnosis of this condition is critical. REFERENCE #1: Vashisht P, Bansal P, Goyal A, Hearth Holmes MP. Sweet Syndrome. 2020 Sep 14. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–. PMID: 28613704. DISCLOSURES: No relevant relationships by Rabia Anees, source=Web Response No relevant relationships by Rosa Arancibia, source=Web Response No relevant relationships by Andrew Doodnauth, source=Web Response No relevant relationships by Khawaja Omar, source=Web Response