1. Phacomatosis Pigmentovascularis: A New Syndrome? Report of Four Cases
- Author
-
Ramon Ruiz-Maldonado, Arturo Lopez, Lourdes Tamayo, Gabriela Brawn, and Amelia M. Laterza
- Subjects
Male ,Pathology ,medicine.medical_specialty ,Skin Neoplasms ,Eye Diseases ,Phacomatosis pigmentovascularis ,Eye disease ,Diagnostico diferencial ,Dermatology ,Phakomatosis ,medicine ,Humans ,Brain Diseases ,business.industry ,Infant ,Syndrome ,medicine.disease ,Hyperpigmentation ,Phakomatosis pigmentovascularis ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Capillary vessels ,Nevus flammeus ,Female ,sense organs ,medicine.symptom ,Hemangioma ,business ,Pigmentation Disorders - Abstract
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 clinicaily 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, tnelanocytes of normal aspect were present in the middle and deep der-mis. The characteristic oculocutaneous pigmentation probably represented a noncoincidental association. The term phacomatosis pigtnentovascuiaris seems appropriate for this apparently new neurocutaneous syndrome.
- Published
- 1987
- Full Text
- View/download PDF