Leri pleonosteosis
# Leri pleonosteosis

Leri's pleonosteosis is a rare rheumatic condition. It was first described by the French physician Leri in 1921.

## Presentation

The clinical features of this condition include

* Flattened facial features
* Flexion contractures of the interphalangeal joints of hand and foot.
* Limited motion of multiple joints
* Short broad metacarpals, metatarsals and phalanges

Thickening of the skin may occur in a fashion similar to that occurs in scleroderma. The thumbs may be angled in a lateral direction (valgus deformity). The knees may be angled backwards (genu recurvatum). Abnormalities of the upper spinal cord may also occur.

## Genetics

It is inherited in an autosomal dominant fashion.The pathogenesis of this condition appears to be due to over expression of two genes - GDF6 and SDC2. These genes are located on the long arm of chromosome 8(8q22.1).

