VEXAS (Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently described adult-onset inflammatory and hematological disorder.[[1]] It is caused by a somatic mutation in the I UBA1 i gene on the X chromosome, which encodes the major E1 ubiquitin-activating enzyme. Any associated macrocytic anemia, thrombocytopenia, or vascular thrombosis in a male patient above 50 years of age should raise suspicion for VEXAS syndrome. Dermatologists may be the first to encounter VEXAS syndrome patients as almost 90% of patients have been reported to have cutaneous manifestations, with a majority presenting initially due to cutaneous disease.[[3]] In this article, we describe the key clinical manifestations of VEXAS syndrome and offer diagnostic clues that will aid in prompt diagnosis and reduce the associated mortality. [Extracted from the article]