Description of the disease * EnglishPolish Pobierz sekcję do PDF 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 *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl