Phacomatosis pigmentovascularis: a new syndrome? Report of four cases.

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 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.

• Authors: R Ruiz-Maldonado, L Tamayo, A M Laterza, G Brawn, A Lopez
• Journal: Pediatric dermatology (Pediatr Dermatol) Vol. 4 Issue 3 Pg. 189-96 (Nov 1987) ISSN: 0736-8046 [Print] United States
• PMID: 3422849 (Publication Type: Case Reports, Journal Article)

Topics

• Brain Diseases (complications)
• Child, Preschool
• Eye Diseases (complications)
• Female
• Hemangioma (complications)
• Humans
• Infant
• Male
• Pigmentation Disorders (complications)
• Skin Neoplasms (complications)
• Syndrome