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Псевдоксантома еластичноподібний папілярно-дермальний еластоз

ORPHA:228293· ICD-10 L98.8· Pseudoxanthoma elasticum-like papillary dermal elastolysis

Визначення(English summary)

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.

Поширеність
Unknown
Успадкування
Not applicable
Вік початку
Adult