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.

Epidemiology

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

Clinical presentation

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.

Pathology

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.

Radiographic features

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.

History and etymology

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.

Differential diagnoses

• osteoarthritis
• CPPD
• rheumatoid arthritis
• septic arthritis / osteomyelitis