Korean J Dermatol.
1999 Aug;37(8):1069-1073.
A Case of Phakomatosis Pigmentovascularis Type IIb Associated with Perirenal Lipomatosis
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- 1Department of Dermatology, Chonnam University Medical School, Kwangju, Korea.
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.
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