| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 6069494 | Journal of the American Academy of Dermatology | 2016 | 5 Pages |
Abstract
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.
- 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.
Keywords
Related Topics
Health Sciences
Medicine and Dentistry
Dermatology
Authors
Dominique C. MD, Amanda K. MD, Patrycja MS, CRNP, Deborah L. MD, Edward W. MD, MHSc,