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Dermatosparaxis

Individuals demonstrate severe skin fragility and bruising. Wound healing is not impaired and the scars are not atrophic, skin texture is soft and doughy. Sagging, redundant skin is evident. The redundancy of facial skin results in an appearance resembling cutis laxa. Large hernias (umbilical, inguinal) may also be seen.

Dermatosparaxis Type EDS is caused by a deficiency of procollagen I N-terminal peptidase. Inheritance: Autosomal recessive.