Acquired pseudoxanthoma elasticum

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Synonyms: Acquired Gronblad-Strandberg-Touraine syndrome | Acquired PXE

A rare acquired dermis elastic tissue disorder characterized by clinical and histopathologic evidence of pseudoxanthoma elasticum in the absence of a family history or specific mutation. Patients present with predominantly cutaneous manifestations consisting of yellowish papules which coalesce into large plaques and are most commonly localized on the neck axillae groin and flexural surfaces. Skin biopsy shows accumulation of clumped calcified elastic fibers in the mid-dermis. Reported underlying factors include previous liver transplantation exposure to penicillamine or concomitant beta-thalassemia.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version July 2024

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