Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Becker nevus syndrome


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.

Symptoms

Newline Maker

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

The Human Phenotype Ontology provides the following list of signs and symptoms for Becker nevus syndrome. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Abnormality of adipose tissue 90%
Amyotrophy 90%
Generalized hyperpigmentation 90%
Micromelia 90%
Supernumerary nipples 90%
Breast aplasia 50%
Pectus carinatum 50%
Pectus excavatum 50%
Abnormality of the ribs 7.5%
Abnormality of the scrotum 7.5%
Abnormality of the tibia 7.5%
Asymmetric growth 7.5%
Kyphosis 7.5%
Scoliosis 7.5%
Spina bifida occulta 7.5%

Last updated: 9/2/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


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.