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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