Every year, the ACR conducts a case-based image competition. Images are submitted for peer review. The winning images are displayed at ACR Convergence and winners receive other benefits, as well. This year, the overall winning images were submitted by Madhuri Challa, MD, of Hyderabad, Telangana, India.
Presentation
These are the representative clinical and radiological images of a 2-year-old child who had presented with fever from just 8 months old. He had skin rash, knee pain and swelling, and organomegaly. He was diagnosed with neonatal onset multisystem inflammatory disease (NOMID), with a likely pathogenic variant in the NLRP3 gene exon 4, C926 T >C in whole exome sequencing.
Figures A and B illustrate the typical non-pruritic, urticarial rash over the back and face, respectively. Figure C shows bony overgrowth and deformity at the knee joint. Figure D shows the corresponding knee radiograph (lateral view) with patellar overgrowth and premature ossification.
NOMID
According to the National Organization for Rare Diseases, NOMID is a rare, systemic, inflammatory condition characterized by fever, rash, joint symptoms, and central nervous system (CNS) symptoms.1 The hallmark of NOMID is onset during early infancy, usually before 6 months of age. Severity varies by child. Early diagnosis and prompt treatment is important to prevent severe complications of the disease and improve life expectancy.
NOMID is the least common and most severe form of the cryopyrin-associated periodic syndromes (CAPS) caused by mutations in the CIAS1/NLRP3 gene. All of these syndromes are characterized by fever, rash and musculoskeletal pain.
The Rheumatology Image Library is a highly accessed teaching resource, and images from the ACR’s annual contest are added to it, helping keep it dynamic and current. Look for the People’s Choice regional winners in future issues and on our website.


