Preferred Label : pseudoxanthoma elasticum;
RADLEX synonyms : Grönblad–Strandberg syndrome; Gronblad–Strandberg syndrome;
Definition : A rare, progressive inherited disorder resulting from extensive basophilic degeneration
of elastic tissue, usually presenting after puberty and involving the skin, eye, and
cardiovascular system. Characteristic manifestations are small, circumscribed yellowish
patches at sites of considerable movement of the skin, angioid streaks in the retina,
and a tendency towards hemorrhage and arterial insufficiency. [MeSH];
UMLS CUI (RADLEX) : C0033847;
Origin ID : RID3271;
UMLS CUI : C0033847;
Currated CISMeF NLP mapping
- Semantic type(s)
- UMLS correspondences (same concept)
A rare, progressive inherited disorder resulting from extensive basophilic degeneration
of elastic tissue, usually presenting after puberty and involving the skin, eye, and
cardiovascular system. Characteristic manifestations are small, circumscribed yellowish
patches at sites of considerable movement of the skin, angioid streaks in the retina,
and a tendency towards hemorrhage and arterial insufficiency. [MeSH]
