Leiner-Moussou Disease

Leiner-Moussou disease is a rare hereditary connective tissue disease characterized by benign hyperelastosis of the skin, joints and internal organs.

The disease was first described in 1871 by the Austrian pediatrician K. Ledner and in 1926 by the French pediatrician A. Moussou.

The cause of the disease is a mutation in the genes responsible for the synthesis of the proteins elastin and fibrillin, resulting in a disruption of the structure and properties of connective tissue.

Clinical manifestations:

1. hyperelasticity of the skin - when pressed, the skin gathers into folds that do not smooth out

2. flexion contractures of the joints due to hyperelasticity of the ligaments

3. hyperelasticity of the walls of blood vessels and internal organs, which leads to their stretching and deformation

4. tendency to hernia

5. often arterial hypotension

Treatment is mainly symptomatic - surgical correction of contractures and hernias. The prognosis for life and health is favorable.