| PXE | pseudoxanthoma elasticum |
|---|
| PXE | Pseudoxanthoma Elasticum |
|---|
| pseudoxanthoma elasticum | <dermatology> A rare disorder of degeneration of the elastic fibres with tiny areas of calcification in the skin, back of the eyes (retinae), and blood vessels. Pseudoxanthoma elasticum is inherited from the parents, either as an autosomal recessive or as an autosomal dominant trait. It typically causes yellow-white small raised areas in the skin folds, often appearing in the second or third decades of life. These skin abnormalities frequently appear on the neck, armpits, and other areas that bend a great deal (referred to as flexure areas). The face is not affected. There are often see abnormalities in the retina called angioid streaks, which are tiny breaks in the elastin-filled tissue there. These eye abnormalities can lead to blindness. Other areas that can be affected in pxe include the heart which can be affected by atherosclerosis and mitral valve prolapse. Small blood vessels are abnormally fragile in patients with pxe because the blood vessel walls contain elastin and are weakened. This can lead to abnormal bleeding in such areas as the bowel and, very rarely, the uterus. Impairment of circulation to the legs can lead to pains in the legs while walking (claudication). Inheritance: ausomal recessive and autosomal dominant Abbreviation: PXE (12 Dec 1998) |
|---|
| pseudoxanthoma cell | <cell biology> Relatively large phagocytic cell's (macrophages) that contain numerous small lipid vacuoles or haemosiderin (or both), in organizing haemorrhagic or inflammatory lesions. (05 Mar 2000) |
|---|
Synonyms : Gronblad Strandberg Syndrome, Syndrome, Gronblad-Strandberg
| pseudoxanthoma elasticum |
a rare, progressive disorder with autosomal recessive and dominant forms, usually appearing after puberty with skin, eye, and cardiovascular manifestations, most of which result from basophilic degeneration of elastic tissue. Symptoms include small yellow cutaneous macules and papules that merge to form plaques, mainly in flexural areas; lax, inelastic, redundant skin; angioid streaks in the retina; arterial insufficiency in the lower extremities; premature calcification of peripheral arteries; reduced arterial pulses; symptoms of coronary insufficiency, hypertension, and mitral valve prolapse; and gastrointestinal and other hemorrhages. Called also nevus elasticus.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
|---|
제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
|---|
제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
|---|