Cutis laxa, autosomal recessive, type 1B

Cutis laxa, autosomal recessive, type 1B

Definition

An autosomal recessive cutis laxa type I characterized by disturbed elastic fiber formation resulting in severe systemic connective tissue abnormalities that has material basis in homozygous or compound heterozygous mutation in the EFEMP2 gene on chromosome 11q13.

Also known as ARCL1B — per MONDO

Also identified as