Pseudoxanthoma elasticum-like papillary dermal elastolysis

Orpha code: 228293OMIM code:

Definition

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare, acquired, idiopathic dermal tissue disorder characterized by numerous, asymptomatic, 2-3 mm, yellowish, non-follicular papules that tend to converge into cobblestone-like plaques which are distributed symmetrically over the posterior neck, supraclavicular region, axillae, and sometimes abdomen. Unlike PXE, these skin lesions show select elimination (absence or marked loss) of elastic fibers in the papillary dermis and there is no systemic involvement.

Disease data
Classification

Disease

Synonyms
PXE-like papillary dermal elastolysis
Brodawkowata elastozytoza skóry podobna do PXE
ORPHA code
228293
OMIM code
-
ICD10 code
L98.8
ICD11 code
EE41.Y

No additional description.

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