VEXAS, a new adult-onset autoinflammatory syndrome .

[Shell567]

Image by @MithuRheum, Twitter.

Somatic Mutations in UBA1 and Severe Adult-Onset Autoinflammatory Disease (VEXAS SYNDROME). "Most of these 25 patients (male) met clinical criteria for an inflammatory syndrome (relapsing polychondritis, SWEET'S SYNDROME, polyarteritis nodosa, or giant-cell arteritis) or a hematologic condition (myelodysplastic syndrome or multiple myeloma) or both." nejm.org/doi/full/10.1056/N...

[Shell567 Administrator Sweet's Syndrome UK]

VEXAS Syndrome, DermNet NZ, Sept 2021.

"Cutaneous features:

Polychondritis — involving the nose and/or ear; sparing the airways and ribs

Neutrophilic dermatoses — resembling acute febrile neutrophilic dermatosis (SWEET'S SYNDROME); tender, red-violaceous, firm, pigmented or purpuric papules, nodules, and plaques which can be umbilicated

Vasculitis — resembling livedo racemosa, polyarteritis nodosa, or giant cell arteritis." dermnetnz.org/topics/vexas-...