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Papers of the Week

2019 Jul 10

Pediatr Rheumatol Online J



Testicular ischemia in deficiency of adenosine deaminase 2 (DADA2).


Clarke K, Campbell C, Omoyinmi E, Hong Y, Al Obaidi M, Sebire N, Brogan PA
Pediatr Rheumatol Online J. 2019 Jul 10; 17(1):39.
PMID: 31291964.


Deficiency of adenosine deaminase 2 (DADA2) is a rare autosomal recessive autoinflammatory condition. Recognised features include vasculitis predominantly affecting medium sized vessels, livedoid skin rash, central and peripheral nervous system involvement, variable degrees of immunodeficiency, and marrow failure, amongst other clinical presentations. We present the case of a six year old male with DADA2 who presented with acute testicular ischaemia secondary to vasculitis, the first such description in DADA2.