Acquired pseudoxanthoma elasticum

Orpha code: 228247OMIM code:

Definition

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.

Disease data
Classification

Disease

Synonyms
Acquired Gronblad-Strandberg-Touraine syndrome
Nabyte PXE
Nabyty zespół Gronblada, Strandberga i Touraine'a
Acquired PXE
ORPHA code
228247
OMIM code
-
ICD10 code
L98.8
ICD11 code
EE41.Y

No additional description.

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