Back issues No.2 - 1998 / Case Report  

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Phakomatosis Pigmentovascularis Type Iib with Right Kidney Agenesis
Ching-Yuang Huang, Yu-Yun Lee, Ching-Yie Wu
Dermatol Sinica 16: 147-152, 1998

We report a rare case of phakomatosis pigmentovascularis (PPV), type Iib, in association with right kidney agenesis. An 8-year-old boy was admitted to our hospital for urinary tract infection. Extensive port-wine stain, aberrant Mongolian spots, leg-length discrepancy, pelvic obliquity, scoliosis and bilateral melanosis oculi bulbi were noted. Further investigation revealed right kidney agenesis which has never been observed in PPV.

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