Orphanet: Thiemann disease, familial form

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Thiemann disease, familial form

Disease definition

Thiemann disease is a very rare genetic necrotic bone disorder characterized clinically by painless swelling of the proximal interphalangeal joints associated with osteonecrosis of epiphyses followed by osteoarthritic changes, with onset before 25 years of age and often a benign course.


Classification level: Disorder
  • Synonym(s):
    • Aseptic necrosis of phalangeal epiphyses
    • Osteochondrosis of phalangeal epiphyses
  • Prevalence: -
  • Inheritance: -
  • Age of onset: Adolescent
  • ICD-10: M93.2
  • OMIM: 165700
  • UMLS: -
  • MeSH: -
  • GARD: 4131
  • MedDRA: -
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