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Types of Cutis Laxa

 Cutis laxa is a hereditary and sometimes acquired connective tissue disorder that causes the skin to stretch and hang in loose folds. Mild cases of the disorder only affect the skin, but more severe cases can affect the internal organs.

Types of Cutis laxa include sex-linked cutis laxa, autosomal recessive cutis laxa type 1, autosomal recessive cutis laxa type 2, autosomal dominant cutis laxa and acquired cutis laxa. Sex-linked cutis laxa is caused by a defective gene on the x-chromosome. Autosomal recessive cutis laxa type 1 is caused by a defective gene on chromosome 5. Autosomal recessive cutis laxa type 2 is inherited as a recessive trait. Autosomal dominant cutis laxa is caused by a defective gene on an autosomal chromosome. Acquired cutis laxa usually follows a severe illness and is characterized by fever, inflammation, severe skin rash, injury to nerves that control blood vessel dilation and contraction or an autoimmune condition.

The most noticeable symptom of Cutis laxa is loose skin. Other symptoms include mild mental retardation, joint abnormalities, frequent loose stools, urinary tract blockages, deficiencies in Lysol oxidase, missing elastic fibers, premature aging, pulmonary emphysema, diverticulas in esophagus, duodenum and bladder, lax and dislocated joints, tortuous arteries, hernias, delayed joining of cranial bones, curvature of the spine, flat feet and excessive tooth decay.

Similar disorders include Ehlers-Danlos syndrome, Marfan syndrome, Menkes disorder, Cutis hyperelastica, Turner’s syndrome and neurofibromatosis.

The disorder is easy to diagnose because of the loose skin, but there is no cure and treatment is limited. The complications of the disorder can be treated, but the disorder itself cannot be treated. Cosmetic surgery can be done to reduce the loose skin, but this is usually only a temporary fix.

There is no way to prevent the disorder and the cause is unknown. Possible causes include abnormal copper metabolism or copper deficiency, decreased serum elastase inhibitor level, low lysol oxidase activity, increased elastase activity, post inflammatory elastolysis, immune-mediated mechanism and decreased elastin gene expression.

If the condition is mild a person with Cutis laxa may be able to lead a normal life with a normal life expectancy, but severe cases can be fatal.
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By: C.Olvera
Published: 02/22/08


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