<unk> , or<unk>  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. The lesions have a variable severity. However, it has been subsequently found that mesodermal and endodermal tissues too are involved. These diseases often involve a disorder of non-cancerous skin cells, melanocytes, which produce pigment appearing as moles, birthmarks or growths on the surface of the skin or just beneath, which can be rough, flat or raised. These dermal growths, including port wine stains, moles, discolouration, café au lait spots and hyperpigmentation, are often composed of blood vessels which can cause functional or cosmetic problems, as well as benign tumours located on the surface or just below of the skin. These physical symptoms are caused by systemic tumour-like growths of the eye, brain, skin, internal organs and bones. The tendency of the condition to involve multiple bodily systems means the conditions are also known as ocular-neurocutaneous syndromes, with about half of people with PPV experiencing systemic symptoms, often suffering from neurological, ocular or muscular abnormalities. These conditions are regularly present at birth, though there are instances of the syndromes appearing later on in life.
Phakomatosis
Lipodermatosclerosis
Porokeratosis
Hypodermyasis
Universal angiomatosis
Verruciform xanthoma
Boomerang dysplasia
Granulomatous slack skin