Cutis laxa is a rare genetic connective tissue disorder that manifests as wrinkled, loose skin. What are the symptoms ? What is it due to? What is the treatement ? An operation ? What life expectancy?
If a child has droopy, wrinkled skin, he is probably suffering from cutis laxa, a rare connective tissue disease. This may be due to the malfunction of a gene and so be hereditary or follow an illness causing a lot of fever. If it can be difficult to live with from an aesthetic point of view, it can however be much more serious if it reaches the noble organs. What are the symptoms ? What complications? Elements of answers with Dr. Marc Perrussel, dermatologist.
Definition: what is cutis laxa?
The cutis laxa is a rare genetic connective tissue disease which causes a lack or poor quality of elastic fibers. The skin therefore looks saggy and wrinkled in patients even very young ones.
What are the symptoms of cutis laxa?
The main symptom is therefore loose skin with an early aged appearance. But other symptoms may appear, if the cutis laxa reaches the internal organs:
- heart trouble,
- 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 causes cutis laxa?
“Cutix laxa is usually caused by a faulty gene. In this case, it is mainly hereditary. But cutis laxa can also occur, especially in children, after an illness that would have caused fever and inflammation of certain organs”, explains Dr. Marc Perrussel, dermatologist.
The clinical examination allows to suspect a cutis laxa. Especially if there are identical cases in the family. A biopsy can also help to find the absence of elastin in the skin.
What is the treatment for cutis laxa?
Concerning the skin, no treatment exists except reconstructive surgery but its effects can only be temporary. If the Cutis Laxa affects other organs, then the complications will be treated appropriately.
When to consider surgery?
“Surgery can be considered when the impact of cutis laxa is too great in everyday life. We help patients go through stages but 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 heart, lungs, intestines or arteries. Heart, lung 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.
Thank you to Dr. Marc Perrussel, dermatologist and member of the national union of dermatologists-venerologists.