A Case of Phakomatosis Pigmentovascularis Type IIb Associated with Perirenal Lipomatosis.
- Author:
Sham Sik SHIN
1
;
Seong Jin KIM
;
Seung Chul LEE
;
Young Ho WON
Author Information
1. Department of Dermatology, Chonnam University Medical School, Kwangju, Korea.
- Publication Type:Case Report
- Keywords:
Perirenal lipomatosis;
Phakomatosis pigmentovascularis
- MeSH:
Adolescent;
Female;
Hemangioma;
Humans;
Lipomatosis*;
Mongolian Spot;
Neurocutaneous Syndromes*;
Nevus;
Nevus of Ota;
Nevus, Pigmented;
Port-Wine Stain;
Sturge-Weber Syndrome
- From:Korean Journal of Dermatology
1999;37(8):1069-1073
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Phakomatosis pigmentovascularis is a distinctive association of cutaneous hemangiomas and melanocytic nevi. The hemangiomas consist of extensive nevus flammeus, while the melanocytic lesions may be aberrant mongolian spots, nevus spilus, nevus of Ota, and nevus of Ito. Four types of the disorder have been recognized, each of which are subgrouped further by the abscence (type a) or presence(type b) of systemic organ involvement. We present a case of phakomatosis pigmentovascularis associated with Klippel-Trenaunay-(Parkes)-Weber sydrome, Sturge-Weber syndrome, nevi of Ota and Ito and perirenal lipomatosis in a 13-year-old girl. To the best of our knowledge, the coexistance of the perirenal lipomatosis in phakomatosis pigmentovascularis has not been reported.
