DADA2 is an autosomal recessive syndrome characterized by recurrent fever, early-onset stroke, livedo racemosa, PAN, and hepatosplenomegaly.

Preliminary experience suggests anti–TNF-α therapy may prevent further CVA, improve cutaneous PAN, and improve hepatobiliary function, but further study is needed.

Early identification and confirmation of mutations in the CECR1 gene can potentially decrease morbidity from this disease by guiding selection of appropriate therapy early in the course of disease.