Cutis laxa is a rare genetic connective tissue disease that manifests as wrinkled and sagging skin. What are the symptoms? What is this due to? What is the treatment? An operation? What life expectancy?
If a child has sagging, wrinkled skin, is that he probably suffers from cutis laxa, a rare connective tissue disease. This may be due to gene dysfunction and thus be hereditary or follow an illness causing high fever. Although it can be difficult to live with from an aesthetic point of view, it can however be much more serious if it reaches the internal organs such as the brain, heart, lungs, liver, pancreas, small intestine and kidneys. What are the symptoms ? What complications ? Some answers with Dr. Marc Perrussel, dermatologist.
Definition: what is cutis laxa?
The cutis laxa is a rare genetic connective tissue disease which generates a lack or poor quality of elastic fibers. The skin therefore has an appearance flabby and wrinkled in patients even those who are very young.
What are the symptoms of cutis laxa?
The main symptom is therefore sagging skin with an early aged appearance. But other symptoms can appear if the cutis laxa reaches the internal organs:
- heart problems,
- lung disorders
- digestive disorders
Usually the symptoms are visible from birth but in some cases, they can develop during childhood, adolescence or even adulthood after certain types of lymphoma.
What is the cause of cutis laxa?
“Cutix laxa is usually caused by a defective gene. In this case, it is mainly hereditary. But cutis laxa can also occur, particularly in children, after an illness which would have caused fever and inflammation of certain organs”, explains Dr. Marc Perrussel, dermatologist.
Clinical examination allows us to suspect cutis laxa. Even more so if there are identical cases in the family. A biopsy can also help detect possible abnormalities in the elastic fibers of the connective tissue.
What is the treatment for cutis laxa?
Concerning the skin, no treatment exists except reconstructive surgery but its effects can only be temporary. If Cutis Laxa affects other organs, then the complications will be treated appropriately.
When to consider surgery?
“Surgery may be considered when the impact of cutis laxa is too significant in everyday life. We help patients overcome milestones but we always warn them that the effects will be temporary“, explains the dermatologist.
What are the possible complications of cutis laxa?
Cutis laxa can also affect noble organs such as the heart, lungs, intestines or even arteries. Cardiac, pulmonary or digestive disorders may be present.
What is the life expectancy in case of cutis laxa?
Life expectancy depends on the internal organs affected because heart or lung conditions can be fatal.
Thanks to Dr Marc Perrussel, dermatologist and member of the national union of dermatologists-venerologists.
