J Korean Pediatr Soc.  1994 Aug;37(8):1156-1161.

A Case of Phakomatosis Pigmentovascularis

• Park SM
• Park KI
• Joo HJ
• Namgung R
• Lee C
• Han DG
• Chun SI

Abstract

In 1947, Ota et al. first reported a case of phakomatosis pigmentovascularis and defined a subgroup of this disease having a congenital generalized hemangioma and pigmented lesions including a mongolian spot-like lesion, nevus pigmentosus and nevus of Ota. We experienced a case of phakomatosis pigmentovasularis type IIb in a 10-day old male baby, who since birth, had generalized nevus flammeus and blue spots, syndactyly between 2nd and 3rd toes in both feet, and dilated collecting system in left kidney.

MeSH Terms

Foot
Hemangioma
Humans
Kidney
Male
Neurocutaneous Syndromes*
Nevus
Nevus of Ota
Parturition
Port-Wine Stain
Syndactyly
Toes