VEXAS Syndrome: A Review for the Inpatient Dermatologist

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a rare autoinflammatory disease process that generally affects older men and demonstrates high morbidity and mortality. As patients are often hospitalized for the recurrent fevers, constitutional symptoms, and dermatologic findings, dermatologists should be aware of cutaneous findings that would raise suspicion for VEXAS syndrome.

Characterized initially by the National Institute of Health in 2020, VEXAS syndrome is characterized by a UBA1 mutation. Cutaneous findings, including neutrophilic dermatoses, vasculitides, and relapsing polychondritis are frequent manifestations of VEXAS syndrome. Diagnosis is established when patients present with clinical features in two different organ systems known to be associated with VEXAS syndrome (rheumatologic, hematologic, dermatologic, pulmonary), and supportive laboratory test abnormalities (anemia and/or thrombocytopenia).

Patients with VEXAS syndrome have a 5-year survival rate ranging from 50–63%. Common dermatologic findings include Sweet syndrome-like lesions, relapsing polychondritis, and cutaneous small vessel vasculitis with palpable purpura. In men in their 5th decade or life or older, who present with recurrent fevers, neutrophilic dermatoses, and/or associated hematologic, rheumatologic or pulmonary findings, clinicians should consider the possibility of VEXAS syndrome.