Definition
Definition
Darier's pseudoxanthoma elasticum is a disease of skin characterized by small breeding farms white or yellowish in color. Their number is significant, and they sometimes group together to form a relatively large patch of purple coloring.
Generalities
Classically, pseudoxanthoma elasticum gives the impression of goosebumps. The skin appears thickened, soft and loose.
This dermatosis associates with stretch marks (small, scar-like skin striations appearing on very stretched skin).
Classification
There are four Darier pseudoxanthomas elasticum:
- Two are autosomal dominant (it is enough for one of the parents to carry the genetic anomaly for the child to have the disease).
- Two are autosomal recessive (both parents must carry the anomaly at the gene level for the child to have the disease).
Symptoms
Symptoms
Symptoms of Darier's pseudooxanthoma elasticum are:
- It appears mainly on each side of the neck, at the elbow crease, the popliteal fossae (behind the knees) and the armpits (below the junction of the arms and the trunk), which present loose, redundant (superfluous) folds of skin with no elasticity.
- These skin lesions are usually seen in children.
Cause
Cause
The causes of Darier pseudoxanthoma elasticum are:
- This disease of genetic and congenital origin (affecting the fetus during pregnancy) is explained by a rupture of the elastic fibers belonging to the connective tissue (support tissue of the body), and due to degeneration.
- The mechanism is a progressive destruction of a set of elastic fibers in the deep areas of the skin with swelling of these fibers which melt and eventually fragment.
- Calcium deposits can also be seen on the elastic fibers of the skin of the eye and blood vessels. Lesions appear in the flexion areas of the neck (skin folds). These mechanisms explain the appearance of slightly raised, linear yellow spots.
Evolution
Premature
Complications of Darier pseudoxanthoma elasticum are:
- Some patients have mild forms of the disease for which diagnosis is not always easy.
- bruising (bruises) of frequent occurrence.
- Complications may affect the vessels, leading to hypertension (elevation of blood pressure above normal).
- Gastrointestinal bleeding (of the stomach and intestines).
- Cerebral hemorrhages (at brain level).
- A lack of blood circulation in the periphery (at the extremities of the limbs) can lead to claudication (lameness).
- achievement of coronary (angina among others).
- L'elastorrhexis is likely to also affect the eye (Bruch's membrane), and more particularly the choroidal vitreous and retina, causing angiomatous retinal streaks (damage to small vessels). The vitreous is a transparent and viscous liquid, located in a cavity of the eye just behind the lens.