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Phacomatosis pigmentovascularis (PPV) was described first as a syndrome by Ota in 1947. Patients with this syndrome show cutaneous hemangiomas associated with melanotic or epidermal nevi. The hemangiomas consist of partial nevus flammeus, while the melanocytic lesions may be persistent aberrant mongolian spots. The disorders are categorized into four types and each group is subgrouped further by...
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