Which connective tissue abnormalities are characteristic of arthrogryposis multiplex congenita (AMC)?

Updated: Nov 11, 2020
  • Author: Mithilesh Kumar Lal, MD, MBBS, MRCP, FRCPCH, MRCPCH(UK); Chief Editor: Maria Descartes, MD  more...
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Skin webs (pterygia) across joints, with limitation of movement, are common. Skin dimples are common over joints where movement is limited.

Skin may be soft, doughy, thick, or extensible. Subcutaneous fat is decreased or increased. Inguinal, umbilical, or diaphragmatic hernias may be present. Thickness in joints, symphalangism, and abnormalities in tendon attachment and length may also be present.

Associated skin defects include scalp defects, amniotic bands on limbs, and nail defects.

Pretibial linear skin indentation may be a sign of autosomal recessive inheritance or at least a sign for an increased risk of recurrence.

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