/late-onset focal dermal elastosis

late-onset focal dermal elastosis

Rare Disease

MONDO:0016437

Also Known As

PXE-like late-onset focal dermal elastosispseudoxanthoma-like late-onset focal dermal elastosis

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.