[Bi-symptomatic CINCA syndrome with inaugural urticaria and major articular lesions]

Abstract

Background: CINCA syndrome (chronic infantile neurological cutaneous and articular syndrome) observed in young children associates chronic urticaria and rheumatological disorders which may cause deformation of the larger peripheral joints. Neurological signs develop later leading to severe prognosis: chronic meningitis, deafness, mental retardation. We present a bisymptomatic case which was distinctive by the absence of central nervous system involvement after 11 years of course.

Case report: Since the age of six months a female child experienced chronic urticaria with nearly daily episodes. At two years limping was also observed. At nine years, the child had highly deformed knee joints giving a tumoral radiological aspect. The diagnosis of CINCA was made. At the age of 11 years, the child had normal psychomotor development and neurological and biological tests were normal. The knee deformations stablized and the child suffered little from the urticaria.

Discussion: This case of infantile chronic urticaria inaugurated a CINCA syndrome, a condition described in France and termed NOMID (neonatal onset multisystem inflammatory disease) in the English literature. Our case was similar to those reported in the literature showing characteristic skin, biological and rheumatological disorders. The absence of neurological involvement is exceptional and has been confirmed by regular surveillance to 11 years of age. Bone pathology showed a pseudotumoral aspect which has not been described previously.