Chronic infantile neurological cutaneous and articular syndrome: two new cases with rare manifestations

CINCA/IOMID is a systemic inflammatory disorder of unknown aetiology that r esembles congenital infection and systemic juvenile chronic arthritis (ICA) . This disorder is characterized by neonatal onset, persistent rash, ocular inflammatory lesions, and progressive articular and neurological involveme nt. We report two new patients with this syndrome. Both children presented periodic bouts of cutaneous rash, fever, organomegaly, articular involvemen t with typical radiological features, and developmental delay. One of the p atients presented neonatal jaundice and elevation of liver enzymes; inflamm atory infiltrates were observed in the liver biopsy. The other patient show ed retinal vasculitis detected at age 18 mo on fundoscopy and fluorescent a ngiography. Therapy with azathioprine was associated with prolonged remissi on or this complication. In both cases, the disease was diagnosed after som e delay. Conclusion: Early hepatitis and retinal vasculitis are rare features of CIN CA/IOMID that may help differentiate this syndrome from IRA. Azathioprine m ay have induced the remission of vasculitis in one case.