Mauclaire disease

Mauclaire disease, also known as Dietrich disease, is a rare disease and refers to osteonecrosis of the metacarpal heads, which usually appears between ages of 13 and 18 years 1,2. This condition may lead to early closure of epiphyseal growth plate and growth disturbance of the involved finger.

The condition is rare and most often spontaneous but may be associated with trauma, systemic lupus erythematosus or steroid use 3.

Localized pain, swelling and restricted range of motion in the affected metacarpophalengeal joint are the main clinical presentations 5. Any metacarpal may be affected however the long metacarpals (2nd and 3rd) are most commonly involved 4.

The pathologic changes are equivalent to those of avascular necrosis of other bones. There is disruption of critical blood supply leading to bone infarction, central necrosis and surrounding hyperemia. Microfractures ensue resulting in flattening and deformity of the bone surface.

In plain radiography of the hand flattening of the metacarpal heads, widening of the joint space and signs of deforming osteoarthritis may seen at initial images 6,7

Osteochondral fragments and periostitis with cortical thickening of the metaphyses are characteristic.

It was first described by Mauclaire in 1927. This was erroneously described by Maes et al. as the Dietrich disease in 1932 due to obscured referencing 8.

Article information

rID: 23410
Synonyms or Alternate Spellings:
  • Dietrich's disease
  • Dietrich disease
  • Osteonecrosis of the metacarpal heads
  • Aseptic osteonecrosis of the head of the metacarpal
  • Avascular necrosis of the metacarpal head
  • AVN of the metacarpal head
  • Mauclaire's disease

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Cases and figures

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