How is cutis laxa treated?
Specific therapies for individuals with cutis laxa can include surgery to repair hernias, skeletal malformations or intestinal diverticula. Some individuals elect for plastic (cosmetic) surgery to improve skin symptoms. Results are typically good, but loose, lax skin often recurs.
What kind of disease is cutis laxa?
Cutis laxa is a connective tissue disorder characterized by skin that is sagging and not stretchy. The skin often hangs in loose folds, causing the face and other parts of the body to have a droopy appearance.
Is cutis laxa fatal?
During childhood, some people with cutis laxa develop a life-long lung disease called emphysema, which can make it difficult to breathe. Depending on which organs and tissues are affected, the signs and symptoms of cutis laxa can range from mild to life-threatening.
What causes Acquired cutis laxa?
MACS is an autosomal recessive disorder caused by mutations in the RIN2 gene. Acquired cutis laxa typically occurs in older adults. Although its cause is unknown, it has been observed in some individuals after certain environmental exposures, such as some medications, infections, or autoimmune diseases.
How many people are affected by cutis laxa?
People with cutis laxa have disorders of the connective tissue, causing normally tight elastic tissue to be loose. An estimated 1 in every 2,000,000 babies has cutis laxa. The very rare condition affects an estimated 400 families worldwide. Cutis laxa is often an inherited condition.
What part of the integumentary system does cutis laxa affect?
Cutis laxa is the general name for a group of rare disorders that affects your connective tissue. This tissue gives your muscles, joints, skin, and organs structure. Most types of cutis laxa are inherited and present at birth along with other disorders, but you can develop some forms later in life.
What is Watson’s disease?
Watson syndrome is an autosomal dominant condition characterized by Lisch nodules of the ocular iris, axillary/inguinal freckling, pulmonary valvular stenosis, relative macrocephaly, short stature, and neurofibromas. Watson syndrome is allelic to NF1, the same gene associated with neurofibromatosis type 1.