Rare Immunology News

Disease Profile

Supernumerary nipple

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

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US Estimated

Europe Estimated

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Accessory nipple; Ectopic nipple; Extra nipple

Categories

Congenital and Genetic Diseases

Summary

A supernumerary nipple is a common, minor birth defect that consists of an extra nipple (and/or related tissue) in addition to the two nipples that normally appear on the chest. Most supernumerary nipples do not cause symptoms or complications. They often are small and go undetected. Sometimes they are first noticed during puberty, menstruation, or pregnancy when hormonal changes affect breast tissue. They can be present with no other tissue (polythelia); with some related tissue; or with breast tissue and ducts (then referred to as polymastia). They are usually located along areas of the body known as the "embryonic milk lines," the lines of potentially appearing breast tissue. The embryonic milk lines extend on both sides of the body from slightly above the armpit, down the chest and abdomen, to the inner thighs near the groin. Supernumerary nipples are usually not inherited but familial cases have been reported.[1] Most people do not need treatment, but the nipple and related tissue can be removed for cosmetic purposes or if there is discomfort.[1][2][3]

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Abnormal thorax morphology
Abnormality of the chest
0000765
Abnormality of the kidney
Abnormal kidney
0000077
Autosomal dominant inheritance
0000006
Supernumerary nipple
Accessory nipple
0002558
Do you have updated information on this disease? We want to hear from you.

Treatment

Most people do not need any treatment for a supernumerary nipple.[2] Removal via surgery or another technique may be considered for cosmetic purposes or if there are symptoms that cause discomfort, such as lactation or tenderness.[3]

A thorough work-up for other malformations typically is not needed in a person with a supernumerary nipple who is otherwise healthy. However, a work-up may be recommended if a person has:[1]

  • a supernumerary nipple accompanied by additional minor malformations
  • a prominent ectopic (abnormally-positioned) supernumerary nipple
  • an established family history of supernumerary nipples

Physical exams and mammography of ectopic breast tissue should occur at the frequency recommended for normal breast tissue in any particular person.[1] Supernumerary nipples can undergo similar diseases to normal breast tissue.[3]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Providing General Support

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
    • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 

        References

        1. Nicholas V Nguyen. Supernumerary Nipple. Medscape Reference. April 8, 2016; https://emedicine.medscape.com/article/1117825-overview.
        2. Supernumerary Nipples. MedlinePlus. February 15, 2016; https://medlineplus.gov/ency/article/003110.htm.
        3. Kevin Zheng. Supernumerary Nipple. DermNet NZ. August, 2015; https://www.dermnetnz.org/topics/supernumerary-nipple.

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