Пігментосудинний факоматоз
ORPHA:2875· ICD-10 Q85.8· Phakomatosis pigmentovascularis
Визначення(English summary)
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.
- Поширеність
- <1 / 1 000 000
- Успадкування
- Not applicable
- Вік початку
- Neonatal