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. 2013 Dec 30;66(1):197–202. doi: 10.1002/art.38217

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© 2014 The Authors. Arthritis & Rheumatology is published by Wiley Periodicals, Inc. on behalf of the American College of Rheumatology.

This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

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Clinical features of chronic infantile neurologic, cutaneous, articular syndrome in the patient. A, Persistent urticaria-like skin rash despite canakinumab treatment (4 mg/kg every 8 weeks). At the time this photograph was taken (when the patient was 7 years old), the cryopyrin-associated periodic syndromes Disease Activity Score (DAS) was 11 of 20, and the serum amyloid A (SAA) level was elevated at 154 mg/liter (normal <10). B, Improvement of the skin rash 8 weeks after the dosage of canakinumab was increased to 8 mg/kg every 8 weeks. At the time this photograph was taken, the DAS had declined to 1 of 20, but the SAA level was still elevated at 118 mg/liter. C, Patellar and tibial bony overgrowth, seen on a radiograph obtained when the patient was 7 years old.