BECKER NEVUS SYNDROME

The new term Becker nevus syndrome is proposed for a phenotype charact erized by the presence of a particular type of organoid epithelial nev us showing hyperpigmentation, increased hairiness and hamartomatous au gmentation of smooth muscle fibers, and other developmental defects su ch as ipsilateral hypoplasia of breast and skeletal anomalies includin g scoliosis, spina bifida occulta, or ipsilateral hypoplasia of a limb . The present review includes 23 cases that can be categorized under t his designation. The Decker nevus syndrome usually occurs sporadically . The associated anomalies tend to show a definite regional correspond ence, suggesting a common origin from an early postzygotic mutation. ( C) 1997 Wiley-Liss, Inc.