Orphanet: Progressive pseudorheumatoid arthropathy of childhood

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Progressive pseudorheumatoid arthropathy of childhood

Disease definition

Progressive pseudorheumatoid arthropathy (dysplasia) of childhood (PPAC; PPD) presents as spondyloepiphyseal dysplasia (SED) tarda with progressive arthropathy and is described as a specific autosomal recessive subtype of SED.


Classification level: Disorder
  • Synonym(s):
    • Spondyloepiphyseal dysplasia tarda-progressive arthropathy syndrome
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Childhood
  • ICD-10: Q77.7
  • OMIM: 208230
  • UMLS: C0432215
  • MeSH: C535387
  • GARD: 9184
  • MedDRA: -

Detailed information


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