Infantile onset panniculitis with uveitis and systemic granulomatosis: a new clinicopathologic entity.
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.
University Hospital Gasthuisberg, Leuven, Belgium. carine.wouters@uz.kuleuven.ac.be
Carine H
CH
Wouters
Tammy M
TM
Martin
Dorothee
D
Stichweh
Marilynn
M
Punaro
Trudy M
TM
Doyle
Jinnell A
JA
Lewis
Pierre
P
Quartier
Carlos D
CD
Rose
D001168:Arthritis / Q000150:complications; D006099:Granuloma / Q000150:complications*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D015434:Panniculitis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D013577:Syndrome; D014605:Uveitis / Q000150:complications*
D007166:Immunosuppressive Agents