Mixed nevus vascularis Q82.5

The naevus vascularis mixtus (NVM) is a rare vascular naevus, a combined vascular malformation characterized by the joint occurrence of a naevus flammeus and angiospastic patches of the naevus anaemicus. In addition to localized foci, up to 30% of the body surface can be covered with telangiectatic and angiospastic parts. The mixed vascular nevus is associated with hypotrophy of the soft tissue or abnormalities of the central nervous system. The cerebral vessels are mainly affected.

Mosaic-like GNA11 mutations have been detected.

In molecular genetic terms, it is a binary phenotype resulting from allelic twin formation and is composed of a naevus anaemicus paired with a capillary naevus.

Differential diagnoses of naevus vascularis mixtus (NVM) and NVM syndrome include vascular twin naevi, syndromes with naevus flammeus and other port-wine stains as well as the various forms of phacomatosis pigmentovascularis.

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