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Children's Health Issues
Hereditary Connective Tissue Disorders
Cutis Laxa
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Topics in Hereditary Connective Tissue Disorders
  • Overview of Hereditary Connective Tissue Disorders
  • Cutis Laxa
  • Ehlers-Danlos Syndrome
  • Marfan Syndrome
  • Osteochondrodysplasias
  • Osteogenesis Imperfecta
  • Pseudoxanthoma Elasticum
  • Nail-Patella Syndrome
 
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Cutis Laxa

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Cutis laxa is a rare disorder of connective tissue that causes the skin to stretch easily and hang in loose folds.

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Cutis Laxa

Cutis Laxa

In cutis laxa, the elastic fibers contained in the connective tissue become loose. Sometimes only the skin is affected, but connective tissues throughout the body can be affected. Cutis laxa is usually hereditary. In some kinds of cutis laxa, the abnormal genes cause problems unrelated to connective tissues—for example, mental retardation/intellectual disability.

Cutis laxa can be mild, affecting only a person's appearance, or severe, affecting the internal organs. The skin may be very loose at birth, or it may become loose later. The loose skin is often most noticeable on the face, resulting in a prematurely aged appearance. The lungs, heart, intestines, or arteries may be affected with a variety of severe impairments.

Although symptoms often become noticeable shortly after birth, they may begin suddenly in children and adolescents. Symptoms usually develop after a severe illness involving fever, inflammation of organs such as the lining of the lungs or heart, or erythema multiforme (patches of red, raised skin). In some people, symptoms develop gradually during adulthood.

A doctor can usually diagnose cutis laxa by examining the skin. Sometimes removal of a skin tissue sample for examination under a microscope (biopsy) is necessary. Severe impairments of the heart, lungs, arteries, or intestines can be fatal. Plastic surgery can often improve the appearance of the skin, although the improvement may be only temporary.

Last full review/revision February 2008 by Frank Pessler, MD, PhD; David D. Sherry, MD

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