Late-onset focal dermal elastosis
ORPHA:228227· ICD-10 L98.8
Definition
Late-onset focal dermal elastosis is a rare, acquired, dermis elastic tissue disorder characterized by a pseudoxanthoma elasticum-like papular eruption consisting of multiple, slowly progressive, asymptomatic, 2-5 mm, white to yellowish, non-follicular papules (that tend to form cobblestone plaques) predominantly distributed over the neck, axillae and flexural areas, with no systemic involvement. Skin biopsy reveals a focal increase of normal-appearing elastic tissue in the reticular dermis with no calcium deposits.
- Prevalence
- <1 / 1 000 000
- Inheritance
- Not applicable
- Age of onset
- Adult