Progressive pseudorheumatoid chondrodysplasia simulating juvenile rheumatoid arthritis

Abstract

Four patients from a Muslim family with an inherited skeletal dysplasia are presented. It is more crippling than usual form of spondylo-epiphysial dysplasia tarda and the onset is very early in the life i.e. at 4 years. The disorder is progressive, crippling and has striking clinical resemblance to juvenile rheumatoid arthritis. The striking X-ray appearance is of platyspondyly and irregularities of capital femoral epiphysis. There is absence of any synovitis. Two of these patients are females aged 8 years and 18 years. The third patient is their cousin brother aged 6 years and their uncle who is of 50 years old and crippled.