VEXAS syndrome is a rare entity secondary to UBA1 gene mutations, located on the X chromosome. This mutation generates, as a consequence, a characteristic vacuolation on haematopoietic stem-cells. It is characterized by multiple autoinflammatory and haematologic manifestations, which respond and end up being dependent on corticosteroid treatment. In this publication we present a 2-case series diagnosed at our hospital and make a brief literature review of the published evidence so far.
Keywords: Chondritis; Condritis; Myelodysplastic syndrome; Sweet syndrome; Síndrome de Sweet; Síndrome de VEXAS; Síndrome mielodisplásico; VEXAS syndrome; Vasculitis.
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