Nevus depigmentosus systematicus with partial yellow scalp hair due to selective suppression of eumelanogenesis

We report a Japanese patient with congenital hypomelanosis with a segmental pattern on the left abdomen, whorl-like pattern on the back; mosaic pattern on the chest, right abdomen, and proximal extremities; and with yellow hair on a portion of the scalp. Chemical analysis of the yellow hair revealed decreased eumelanin content, whereas the pheomelanin content was normal.