title: Leri pleonosteosis
text: # 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).
  
  