Early-onset stroke, polyarteritis nodosa (PAN), and livedo racemosa

Key teaching points
- 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.