neurocutaneous syndromes

Phakomatoses, or phacomatosis pigmentovascularis (PPV), is the term used for a group of rare syndromes involving structures arising from the embryonic ectoderm. These are characterised by vascular and pigmentary birthmarks or skin lesions, and often involving multiple organ systems in the body. The term is used to describe the “association of a vascular nevus with an extensive pigmentary nevus”.These multi-system disorders involve the ectodermal structures like central nervous system, skin and eyes. ...