Phakomatosis pigmentovascularis

Definition

A rare skin disease characterized by the co-occurrence of a widespread vascular nevus (typically nevus flammeus) and a pigmentary nevus, potentially associated with a variety of other cutaneous nevi, and with or without extracutaneous (most commonly central nervous system, ocular, or musculoskeletal) involvement. Several subtypes are distinguished based on phenotypic characteristics.

Disease data

Classification

Disease

ORPHA code

2875

OMIM code

ICD10 code

Q85.8

ICD11 code

LD2D.Y

*Soruce