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Infantile onset panniculitis with uveitis and systemic granulomatosis: a new clinicopathologic entity.

Carine H Wouters, Tammy M Martin, Dorothee Stichweh, Marilynn Punaro, Trudy M Doyle, Jinnell A Lewis, Pierre Quartier, Carlos D Rose
Journal of Pediatrics 2007 December
We report on 4 children with infantile-onset lobular panniculitis, high fever, uveitis, and systemic granulomatous inflammation, recruited through the International Registry of Pediatric Granulomatous Arthritis. Neither CARD15 nor CIAS1 mutations were found. Despite immunosuppressive therapy, disease course was progressive. Response to anti-tumor necrosis factor monoclonal antibody in 3 patients is of note.

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