Thiemann disease (also called familial osteoarthropathy of the fingers or osteonecrosis of the base of phalanx) is a non-inflammatory disorder of unknown aetiology and refers to osteonecrosis of the epiphyses of phalanges which leads to deformity of fingers.
Thiemann disease is a rare form of AVN which involved PIP and DIP joints in the second decade of life. The base of the proximal phalanges of the index and middle fingers are usually affected.
Thiemann disease is usually familial ( with an autosomal dominant inheritance pattern). Sporadic cases also reported and tend to occur in males.
Clinical presentation and radiologic appearance of the Thiemann disease correspond closely with those of Mauclaire's disease (osteonecrosis of the metacarpal heads).
An initial painless swelling of the PIP joint with eventual development of the pain and deformity usually is the typical clinical presentation.
Irregularity of the epiphyses of the phalanges is seen. The bones appear broadened, sclerotic, and fragmented. Joint space narrowing may be seen lately.
Treatment is palliative for most cases, in severe disease arthrodesis or arthroplasty may be needed.
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