Cutis Laxa (Elastolysis) Follow-up
- Author: Daniel J Hogan, MD; Chief Editor: Dirk M Elston, MD more...
Discontinuation of cigarette smoking is beneficial to prevent the progression of pulmonary disease.
The most serious complication, which may be life threatening, is cor pulmonale resulting from severe progressive pulmonary emphysema.
In the autosomal dominant form, few, if any, systemic complications occur.
Severe internal complications of the autosomal recessive form include genitourinary and gastrointestinal diverticula, diaphragmatic hernia, and emphysema leading to cor pulmonale and death in the first few years of life.
In acquired cutis laxa (elastolysis), visceral involvement is common and has occurred in all reported patients with onset of acquired disease after the age of 20 years. This may involve the lungs, the gastrointestinal tract, the heart, and the urogenital system with breakdown of elastic fibers and loss of tissue support.
The lifespan of some patients with cutis laxa (elastolysis) may not be significantly decreased. Patients with the autosomal dominant form have a normal life expectancy.
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