THE ANATOMICAL basis for Sturge-Weber-Dimitri syndrome is a hemangiomatous dysplasia in the cephalic region. Since port-wine nevi are frequently found also on the trunk and extremities, the syndrome may be considered as a partial or regional manifestation of generalized neurocutaneous hemangiomatosis. As such, it may be classified along with von Recklinghausen's disease, tuberous sclerosis, and neurocutaneous melanomatosis, for in all of these disorders the skin manifestations indicate the possible presence of changes in the nervous system, as well as in other systems. The clinical features of the syndrome are a vascular nevus of the face, associated with signs of cerebral dysfunction, such as epilepsy, mental deficiency, and hemiplegia, and roentgenographic evidence of sinuous shadows within the cranial cavity. The skin lesion is generally a nevus flammeus of the face or scalp, which often exhibits striking evidence of being confined to the areas of distribution of branches of the trigeminal nerve.
LICHTENSTEIN BW. STURGE-WEBER-DIMITRI SYNDROMECephalic Form of Neurocutaneous Hemangiomatosis. AMA Arch NeurPsych. 1954;71(3):291-301. doi:10.1001/archneurpsyc.1954.02320390021002