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Pseudoxanthoma Elasticum

definition

Generalized disease of connective tissue involving the skin, eyes, and cardiovascular system and resulting from fragmentation and calcification of elastic fibers. Usually presenting after puberty, the characteristic manifestations are small, circumscribed yellowish patches located on the neck, in the axillae or in the inguinal folds. Angioid streaks in the retina, a tendency towards haemorrhage and arterial insufficiency are the most common complications. This disease may be inherited in an autosomal recessive or dominant fashion.

synonyms

Pseudoxanthoma Elasticum, Elastorrhexis Generalisata et Systemica, Grönblad-Strandberg Syndrome

UMLS

ELASTORRHEXIS, SYSTEMIC, OF TOURAINE, ELASTOSIS DYSTROPHICA SYNDROME, Nevus elasticus, Pseudoxanthoma Elasticum, Systematised elastorrhexis, Systematized elastorrhexis

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