CINCA syndrome (NOMID) revealed by urticaria with major joint involvement

Background. CINCA syndrome (chronic infantile neurological cutaneous and ar ticular syndrome) observed in young children associates chronic urticaria a nd rheumatological disorders which may cause deformation of the larger peri pheral joints. Neurological signs develop later leading to severe prognosis : chronic meningitis, deafness, mental retardation. We present a bisymptoma tic case which was distinctive by the absence of central nervous system inv olvement 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 obser ved. At nine years, the child had highly deformed knee joints giving a tumo ral radiological aspect. The diagnosis of CINCA was made. At the age of 11 years, the child had normal psychomotor development and neurological and bi ological tests were normal. The knee deformations stablized and the child s uffered little from the urticaria. Discussion. This case of infantile chronic urticaria inaugurated a CINCA sy ndrome, a condition described in France and termed NOMID (neonatal onset mu ltisystem inflammatory disease) in the English literature. Our case was sim ilar to those reported in the literature showing characteristic skin, biolo gical and rheumatological disorders. The absence of neurological involvemen t is exceptional and has been confirmed by regular surveillance to 11 years of age. Bone pathology showed a pseudotumoral aspect which has not been de scribed previously.