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Genetic and Rare Diseases Information Center (GARD)

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Becker nevus syndrome


Other Names for this Disease

  • Hairy epidermal nevus syndrome
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Overview

What is Becker nevus syndrome?

What are the signs and symptoms of Becker nevus syndrome?

What is Becker nevus syndrome?

Becker nevus syndrome is characterized by the presence of a Becker nevus in association with underdevelopment (hypoplasia) of the breast or other skin-related, muscular, or skeletal defects, all of which usually involve the same side of the body as the nevus (ipsilateral).[1] Specific signs and symptoms in addition to the nevus may include ipsilateral breast hypoplasia; skeletal abnormalities such as hypoplasia of the shoulder girdle, scoliosis, fused ribs, and ipsilateral shortness of the arm; and several other features.[2][1] The condition is thought to be sporadic (occurring in individuals with no history of the condition in the family).[1] Treatment varies depending upon the specific symptoms present and the extent of the condition in the affected individual.[2]
Last updated: 4/25/2012

What are the signs and symptoms of Becker nevus syndrome?

Becker nevus syndrome is characterized by the presence of a Becker nevus in association with underdevelopment (hypoplasia) of the breast and/or other skin-related (cutaneous), muscular, or skeletal defects, all of which usually involve the same side of the body as the nevus (ipsilateral).[1] Breast hypoplasia affects both males and females, but is more noticeable in females.[2] Other muscular and skeletal abnormalities may include absence of the pectoralis major muscle (pectoral); underdevelopment of the muscles of the shoulder girdle; abnormal curvature of the spine (scoliosis); vertebral defects; fused ribs; ipsilateral shortness of a limb; underdevelopment of the teeth and jaws; a "sunken chest" (pectus excavatum) or abnormally prominent chest (pectus carinatum); and other findings.[2][1] Extra (supernumerary) nipples and abnormally sparse hair under the armpit on the affected side of the body have also been reported in some affected individuals.[2]
Last updated: 4/23/2012

References
  1. Wilson H. Y. Lo. Becker Nevus Syndrome. OMIM. May 4, 2000; http://omim.org/entry/604919. Accessed 4/23/2012.
  2. Epidermal Nevus Syndromes. NORD. June 20, 2011; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/862/viewAbstract. Accessed 4/23/2012.


Other Names for this Disease
  • Hairy epidermal nevus syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.