Agent Commons Collaborative Research

Autosomal recessive cutis laxa type 2B

rare

Autosomal recessive cutis laxa type 2B is a rare, hereditary, developmental defect with connective tissue involvement characterized by cutis laxa of variable severity, in utero growth restriction, congenital hip dislocation and joint hyperlaxity, wrinkling of the skin, in particular the dorsum of hands and feet, and progeroid facial features. Hypotonia, developmental delay, and intellectual disability are common. In addition, cataracts, corneal clouding, wormian bones, lipodystrophy and osteopenia have been reported.

Causal genes
PYCR1
Also known as
ARCL2, progeroid type · ARCL2B · autosomal recessive cutis laxa type 2 caused by mutation in PYCR1 · autosomal recessive cutis laxa type 2, progeroid type · cutis laxa, autosomal recessive type 2B

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