Orphanet: Phakomatosis pigmentovascularis

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Disease 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.

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Phakomatosis pigmentovascularis

ORPHA:2875

A summary on this disease is available in Español (2020) Français (2020) Nederlands (2020)

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