Phacomatosis Pigmentopigmentalis: Aberrant Mongolian Spots and Segmental Café Au Lait Macules


Address correspondence to Ronni Wolf, M.D., Dermatology Unit, Kaplan Medical Center, 76100 Rechovot, Israel, or e-mail:


Abstract:  We report a case of an otherwise healthy male newborn, with segmental café au lait spots on his left buttocks and left thigh down to the medial knee, in a nevoid pattern, and an aberrant Mongolian spot on the ipsilateral cheek of the buttocks in a checkerboard pattern. We propose the name phacomatosis pigmentopigmentalis in analogy to phacomatosis pigmentovascularis, and phacomatosis pigmentokeratotica.