Thiemann's disease

Thiemann disease (also called familial osteoarthropathy of the fingers or osteonecrosis of base of phalanx) is a non-inflammatory disorder of unknown etiology and refres to avascular necrosis of the epiphyses of phalanges which leading 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 mey be needed.

Share article

Article information

rID: 23396
Tag: cases
Synonyms or Alternate Spellings:
  • Osteonecrosis of base of phalanx
  • Osteonecrosis of bases of phalanges
  • Familial osteoarthropathy of the fingers

Support Radiopaedia and see fewer ads

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.