Becker's nevus syndrome revisited

Abstract

mented and hypertrichotic skin lesion with unilateral arrangement. This disorder that is today called Becker’s nevus is characterized by the presence of a light or dark brown macule with a sharply outlined but irregular border that resolves into small spots reminiscent of an archipelago. In male patients, the lesion shows increased hairiness after puberty. It tends to be arranged in a checkerboard pattern. 2 Becker’s nevus is fairly common 3 but often overlooked or misdiagnosed. The disorder may become obvious in late childhood or adolescence but for obvious reasons we think that it is always congenital. An association of Becker’s nevus with other developmental anomalies has sometimes been reported. Happle and Koopman 4 reviewed 23 cases and proposed the new term ‘‘Becker’s nevus syndrome’’ for a simultaneous occurrence of Becker’s nevus and unilateral breast hypoplasia or other cutaneous, muscular, or skeletal defects. All of these anomalies tend to show a regional correspondence to the nevus and are mostly ipsilateral. To assess the clinical spectrum of this syndrome in more detail, we present an overview of 55 cases as reported in the literature 3,5-40 and in some personal communications.