/Thiemann disease, familial form

Thiemann disease, familial form

Rare Disease

MONDO:0008142

Also Known As

Osteochondrosis of phalangeal epiphysesaseptic necrosis of phalangeal epiphysesosteochondritis of phalangeal epiphysesTHIEMANN diseaseThiemann epiphyseal diseaseThiemann's diseaseosteoarthropathy of fingers familialosteoarthropathy of fingers, familial

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.