Becker’s Nevus Syndrome (Pigmentary Hairy Epidermal Nevus)

Abstract

Becker nevus syndrome is a phenotype characterised by the simultaneous occurrence of: (1) a circumscribed patch of (light or dark brown) hyperpigmentation with a sharply outlined but irregular border (resolving into small spots reminiscent of an archipelago) and hypertrichosis (with increased smooth muscle bundles) with slight acanthosis (the so-called Becker’s nevus); (2) associated unilateral hypoplasia of one or more of the following: breast, underlying musculature (mostly the shoulder girdle), underlying adipose tissue (lipoatrophy) and limb (usually the arm); and (3) underlying skeletal anomalies including vertebral defects and scoliosis, fused or accessory cervical ribs, pectus excavatum or carenatum, and internal tibial torsion (Danarti et al. 2004, Happle et al. 1997, Sugarman 2004). All of these anomalies tend to show a regional correspondence to the nevus and are mostly ipsilateral (Danarti et al. 2004).