Phacomatosis pigmentovascularis: a new syndrome? Report of four cases.
We examined four patients who had a combination of extensive nevus flammeus, significant oculocutaneous pigmentation, and severe neurologic alterations. All cases were sporadic. The vascular and neurologic alterations were clinically similar to those observed in the Sturge-Weber syndrome. The capillary vessels are ultrastructurally different in phacomatosis pigmentovascularis from those of a nevus flammeus in the Sturge-Weber syndrome; however, melanocytes of normal aspect were present in the middle and deep dermis. The characteristic oculocutaneous pigmentation probably represented a noncoincidental association. The term phacomatosis pigmentovascularis seems appropriate for this apparently new neurocutaneous syndrome.
Ruiz-Maldonado
R
R
Department of Pediatric Dermatology, Instituto Nacional de Pediatria S.S.A., Mexico City, Mexico.
Tamayo
L
L
Laterza
A M
AM
Brawn
G
G
Lopez
A
A
D001927:Brain Diseases / Q000150:complications; D002675:Child, Preschool; D005128:Eye Diseases / Q000150:complications; D005260:Female; D006391:Hemangioma / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D010859:Pigmentation Disorders / Q000150:complications*; D012878:Skin Neoplasms / Q000150:complications*; D013577:Syndrome