Spondyloepiphyseal Dysplasia Tarda with Progressive Arthropathy': In 3 siblings with unusual Association

We report a case series of Spondyloepiphyseal dysplasia tarda with progressive arthropathy SEDT-PA in three siblings. SPEDT-PAis a rare autosomal recessive skeletal dysplasia affecting primarily the articular cartilage with characteristic skeletal abnormalities notably in the spine. First reported by Wynne-Davies et al. who estimated its prevalence to be about 3.4 per million population and an incidence of 1 per 100000 1. live births Because of its striking resemblance to juvenile rheumatoid arthritis it has various synonyms like progressive pseudorheumatoid 2. arthropathy of childhood PPAC and progressive pseudorheumatoid dysplasia PPDA MIM No. 208230 In the present context we present three affected siblings of an inbred south Indian Hindu family with one of them having an unique association of Pierre Robin sequence PRS.