Phakomatosis Pigmentovascularis: Report of a Case


M. Mahroughan, M.D., Amir H. Mehregan, M.D., and David A. Mehregan, M.D.
Pinkus Dermatopathology Laboratory, Monroe, Michigan
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.