Histiocytoid Sweet syndrome (HSS) is a rare histopathologic variant of Sweet syndrome that was first described in 2005 by Requena et al. In contrast to the dense infiltrate of mature neutrophils seen in conventional Sweet syndrome, this variant demonstrates dermal and/or subcutaneous infiltrate with a prominent component of immature myeloid cells resembling histiocytes. (1) A number of publications have since reported similar findings, sometimes in association with hematologic neoplasms. In recent years, an ever increasing number of mutations have been discovered in myelodysplastic syndrome (MDS) and acute myelogenous leukemia (AML), including mutations in isocitrate dehydrogenase 1 (IDH‐1) and isocitrate dehydrogenase 2 (IDH‐2).
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