Back issues No.3 - 2002 / Case Report  

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Phakomatosis Pigmentovascularis Type Iib with Patent Umbilical Vein and Inferior Vena Cava Hypoplasia
Pei-Yu Lo Tien-Yi Tzung
Dermatol Sinica 20: 241-247, 2002

Phakomatosis pigmentovascularis (PPV) is characterized by coexistence of capillary malformation and various melanocytic lesions. Herein, we reported a case of PPV type Iib with patent umbilical vein and, to our best knowledge, this condition has never been described before. This 20-year-old male had nevus of Ota, aberrant Mongolian spots and extensive port-wine stain. He suffered form Sturge-Weber syndrome with left eye blindness, mild mental retardation and epilepsy. Brain computed tomography demonstrated left cerebral calcification and atrophy. In addition, he had Klippel-Trenaunay-Weber syndrome, manifested as hypertrophy of the right lower leg with repeated ulcers and abdominal varicose vein. Angiography of bilateral lower limbs revealed angiomas and vascular malformations. Further studies showed patent umbilical vein and hypoplasia of inferior vena cava.

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