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ABSTRACT: We report two cases of chronic infantile neurological, cutaneous, and articular (CINCA) syndrome. It is a rare congenital multisystemic inflammatory disease characterised by early onset of urticarial skin rash, central nervous system involvement and articular signs. Our two cases were first diagnosed at 13 and 14 years of age, respectively. From birth they presented with typical symptoms of CINCA syndrome, however isolated each symptom was non-specific. After the syndrome was recognised and treatment with anakinra was initiated, most symptoms disappeared.Ugeskrift for laeger 05/2012; 174(22):1537-8.