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.
Wouters
Carine H
CH
University Hospital Gasthuisberg, Leuven, Belgium. carine.wouters@uz.kuleuven.ac.be
Martin
Tammy M
TM
Stichweh
Dorothee
D
Punaro
Marilynn
M
Doyle
Trudy M
TM
Lewis
Jinnell A
JA
Quartier
Pierre
P
Rose
Carlos D
CD
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