All issues > Volume 37(8); 1994

Case Report

J Korean Pediatr Soc. 1994;37(8):1156-1161. Published online August 15, 1994.

A Case of Phakomatosis Pigmentovascularis

Seung Mo SM Park¹, Kook In KI Park¹, Hye Jung HJ Joo¹, Ran R Namgung¹, Chul C Lee¹, Dong Gwan DG Han¹, Soo Il SI Chun²

¹Department of Pediatrics, Yonsei University, College of Medicine, Seoul, Korea
²Department of Dermatology, Yonsei University, College of Medicine, Seoul, Korea

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 anmongolian spot-like lesion, nevus pigmentosus and nevus of Ota. We experienced a case of phakomatosis pigmentovascularis type Iib in a 10-day old male baby, who since birth, had generalized nevus flammeus and blue spots, syndactyle between 2nd and 3rd toes in both feet, and dilated collecting system in left kidney.

Keywords :Phakomatosis pigmentovascularis, Nevus flammeus, Blue spot, Syndactyly, Dilated collecting system