• Dermatosparaxis Type EDS Description

• Dermatosparaxis Type EDS Links

• Medical Journal Abstracts on Dermatosparaxis Type EDS

Dermatosparaxis Type EDS Description

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.

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Dermatosparaxis Type EDS Links

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Medical Journal Abstracts on Dermatosparaxis Type EDS

• Unusual oral findings in dermatosparaxis (Ehlers-Danlos syndrome type VIIC).

• Transforming growth factor-beta induces secretion of activated ADAMTS-2. A procollagen III N-proteinase.

• Cloning and characterization of ADAMTS-14, a novel ADAMTS displaying high homology with ADAMTS-2 and ADAMTS-3.

• Procollagen II amino propeptide processing by ADAMTS-3. Insights on dermatosparaxis.

• Boy with dermatosparaxis (Ehlers-Danlos type VIIC)

• ADAMTS: a novel family of extracellular matrix proteases.

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