Interestingly, an adult-onset autoinflammatory disease associated to UBA1 somatic mutations was recently described in 25 patients, 24% showing MDS and 88% having skin involvement with lesions such as nodules and plaques (compatible with neutrophilic dermatosis or cutaneous vasculitis), periorbital swelling, and severe cutaneous reaction to anakinra injection (21). The gene discussed is UBA1; the disease is Adult onset.