Phakomatosis pigmentovascularis is a rare cutaneous malformation characterized by a combination of melanocytic nevi and nevus flammeus. We report a 16-year-old boy with extensive and predominantly unilateral skin involvement combining features of speckled melanocytic nevi over a background of nevus flammeus. A brief review of the literature and classification of this phakomatosis is presented.
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Mahroughan et al. (1996) studied this question.
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