Frontal whorl. An early sign of peripheral neurofibromatosis?

Abstract

The majority of individuals with straight hair presents a whorl of hair in the parietal area, in front of the fontanelle, on the median line or sometimes laterally on the right or, less frequently, on the left. In the whorl the hair rotates clockwise in most cases. A minority of subjects (2) has a frontal hair whorl. The latter, while being present in normal subjects, is more frequent in some genetic skin disorders (3), in particular those associated with mental retardation. We already reported a frontal hair whorl (1) in peripheral neurofibromatosis (NF1) If future observations confirm an increased incidence of frontal hair whorl in NF1, this could constitute a predictive sign. The latter for its earliness could be helpful to suspect NF1 in children that have only café-au-lait spots.