Background: CINCA syndrome is a clinical syndrome of unclear etiology, characterized by a chronic multi-organ inflammatory process unsusceptible to treatment.

Case Report: An 18-month-old boy was admitted because he suffered, since the age of 2 months, from cutaneous, articular and neurological changes, lymphadenopathy, hepatosplenomegaly, choroiditis and psychosomatic development retardation. These clinical symptoms were unsusceptible to anti-inflammatory and antihistaminic drugs. Rehabilitation was not effective either.

Conclusion: A long-term clinical observation is required before sustained multi-organ changes beginning in early childhood and exclusion of other chronic inflammatory diseases enable the diagnosis of CINCA syndrome.

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http://dx.doi.org/10.1016/s0929-693x(99)80006-2DOI Listing

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