Supernumerary Nipple

Back

Background

Supernumerary nipples (SNs) are a common minor congenital malformation that consists of nipples and/or related tissue in addition to the nipples normally appearing on the chest. SNs are located along the embryonic milk lines. Ectopic SNs are found beyond the embryonic milk lines. The embryonic milk line is the line of potentially appearing breast tissue as observed in many mammals. In humans, the embryonic milk line extends bilaterally from a point slightly beyond the axillae on the arms, down the chest and the abdomen toward the groin, and is generally thought to end at the proximal inner sides of the thighs, although SN has been described on the foot.[1] SNs can appear complete with breast tissue and ducts and are then referred to as polymastia, or they can appear partially with either of the tissues involved.

The classification established by Kajava in 1915 is still valid[2] :

Although this classification is clear, encountering interchangeable terms and misnomers when dealing with the SN complex is not surprising because of the variability in morphologic patterns.

The paucity of descriptions of SNs in medical writings is probably due to its relatively minor clinical significance. However, the subject of SNs has been very popular in the last 2 decades because of the dilemma of possible associated malformations and diseases. The occurrence of SNs has been documented since Roman times and featured in legends and ethnic mythology prior to that time. SNs, and particularly polymastia, were attributed to increased femininity and fertility. Ancient artists depicted the goddess of Artemis of Ephesus and the Phoenician goddess of fertility, Astrate, like other ancient deities, as having row upon row of breasts on their chests.[3] Anne Boleyn, the wife of King Henry VIII, was known to have a third breast. SNs in men were a sign of virility and endowed them with divine powers. Nowadays, film stars expose their SNs in the cinema with this same effect.[3]

The first medical report dates back to 1878 when Leichtenstern estimated the prevalence of SNs to be 1 in 500 (0.2%).[4]

Associations with other diseases

SN features are found in a number of syndromes, but, in most cases, it is probably a chance finding. These syndromes include Turner syndrome, Fanconi anemia, and other hematologic disorders[5] ; ectodermal dysplasia; Kaufman-McKusick syndrome; Char syndrome; Simpson-Golabi-Behmel syndrome; and epibulbar lipodermoids associated with preauricular appendages syndrome.[6] Numerous sporadic publications linked SNs to an association with anomalies or diseases, but such an association is probably only a chance finding. Such associations of SNs include the following:

Publications concerning renal involvement in the presence of SN

In 1979, Méhes drew attention to the association of SNs and other anomalies.[7] The claim that 40% of SNs investigated also had renal involvement was striking. This figure was later corrected to 23-27%.[8, 9] The renal involvement was infectious, a malformation, or neoplastic but mainly due to an obstructive disturbance.

In the following decade, numerous publications supported the claim for a close association of SNs and a renal anomaly, but many others could not find evidence to support such an association, which remains controversial.

The Medscape Reference article Disorders of the Breast may be of interest.

Pathophysiology

Saint-Hilaire in 1836 and Darwin in 1871 advanced the concept of development of the human race from primitive animals; thus, they also considered the supernumerary nipple as an atavistic structure deriving from the milk line of mammals. Similarly, ectopic supernumerary nipple found on the vulva may express an atavistic structure because the breasts of dolphins and whales are in that location, or ectopic supernumerary nipple on the back, the scapula, and the shoulder[32, 33, 34] is reminiscent of the nutria and hutia (rodents) with a similar location of the breasts.

Between the fourth and fifth weeks of embryogenesis, an ectodermal thickening forms symmetrically along the ventral lateral sides of the embryo. This epidermal ridge extends from the axillary region to the inner side of the thigh to form the embryogenic milk (or mammary) line. During the second and third embryogenic months, the glandular elements of the breasts are formed near the fourth and fifth ribs, with regression of the rest of the thickened ectodermal streaks. In the case of failure of a complete regression, some foci may remain to result in a supernumerary nipple. This can develop into a supernumerary complete breast (polymastia) or into any other supernumerary nipple variant according to the Kajava classification.

Epidemiology

Frequency

International

The prevalence of supernumerary nipples varies with different reports. The prevalence is 0.22% in a Hungarian population,[6] 1.63% in black American neonates,[20] 2.5% in Israeli neonates,[21] 4.7% in Israeli Arabic children,[35] and 5.6% in German children.[29] These variabilities are attributed at least partially to differences in geographic regions, ethnic groups, and methodology, including methods of physical examination, as well as the age groups participating in the studies.

Sex

The male-to-female ratio for supernumerary nipples differs in various studies, but, most often, the studies show a male predominance as high as 1.7:1.

History

Usually, the supernumerary nipple remains undetected or asymptomatic. Occasionally, the supernumerary nipple is noticed only when hormonal changes during adolescence, menstruation, or pregnancy cause increased pigmentation, fluctuating swelling, tenderness, or even lactation.

Physical

The supernumerary nipple is often overlooked at the first examination of the neonate. It appears as a small pigmented or pearl-colored mark or as a concave or umbilicated spot (note the image below).


View Image

Supernumerary nipple in a neonate. Courtesy of Dr P. Merlob.

In 75% of patients, it measures no more than 30% of the diameter of the normal nipple (at times no more than 0.2-0.3 cm in diameter). In the other 25% of patients, it is of medium size, as large as 50% of the normal size of the nipple. Rarely, a supernumerary nipple is as large as a normal nipple.[30] It can be mistaken for many other small lesions, most of them hardly noticeable. Note the image below.


View Image

Supernumerary nipple in an adolescent boy. Courtesy of Dr B. Fisher.

Most supernumerary nipples are single, and, when 2 or more (as many as 8) supernumerary nipples are present, they are distributed bilaterally or unilaterally, symmetrically or not. Most supernumerary nipples are located below the regular nipple, while approximately 13% appear above it along the milk line.[22]

When examining adolescent girls (note the image below), the normally developed breast may hide the supernumerary nipple. A number of studies have indicated a preponderance of supernumerary nipples on the right side.[27]


View Image

Supernumerary nipple (bilateral) in an adolescent girl.

For easier detection of the supernumerary nipple, a wet gauze pad is passed along the mammary line (milk line) from the axillary region to the upper part of the thigh on each side. This technique is particularly helpful in the dry and desquamating skin of full-term and postterm infants.[22] When the suggested lesion is concave, folding it between fingers shows a typical wrinkling.

A dermoscopic examination of supernumerary nipple shows a pattern similar to dermatofibroma, with both showing central, white, scarlike areas and a peripheral fine-pigment network. Supernumerary nipple also has a cleftlike appearance in the central area, thus allowing differentiation from dermatofibroma.[36] For a more precise examination one can resort to a reflectance confocal microscopy.[37]

Approximately 5% of supernumerary nipples are ectopic, located outside of the milk line, such as on the back,[34] the shoulder (note the image below),[38] the limbs, the neck, the face, and the vulva and perineum.


View Image

Ectopic supernumerary nipple on the shoulder. Courtesy of Dr B. Fisher.

Causes

Familial cases of supernumerary nipple were recorded as parent-child transmission, including 1 report of a family who had supernumerary nipples in 4 successive generations; therefore, autosomal dominant with incomplete expressivity is the accepted transmission of inheritance.[39]

Histologic Findings

The histologic features of a supernumerary nipple are identical to that of the regular nipple, including hyperpigmentation, slight hyperkeratosis with epidermal thickening, pilosebaceous structure of Montgomery areolar tubercles, smooth muscle bundles typical of the areola, and possible mammary glands and intradermal straight ducts.[40] A significant increase in the number of clear cells of Toker has been found in supernumerary nipple tissue, indicating supernumerary nipple may be a precursor of extramammary Paget disease.[41]

Surgical Care

A protruding (or erectile) supernumerary nipple that causes the patient embarrassment can be easily removed surgically, if desired. Removal using liquid nitrogen cryotherapy has been described.[42] The removal of polymastia or a complete ectopic supernumerary nipple (with breast) is more involved but is indicated in women at high risk of developing breast cancer.[43] To avoid an unsightly scar after the removal of a complete ectopic supernumerary nipple, the tumescent liposuction technique has been suggested.[44, 45]

The Medscape Dermatologic Surgery Resource Center may be of interest.

Consultations

A sporadic supernumerary nipple typically does not present an indication for a thorough workup for other malformations. Exceptions are a supernumerary nipple accompanied by additional minor malformations, a prominent ectopic supernumerary nipple, or an established familial supernumerary nipple. In these instances, consultations for ultrasonography and nephrography investigation are indicated, bearing in mind that kidney and urinary tract pathologic findings can be present but silent.

Prognosis

Most supernumerary nipples can be ignored; occasionally, a cosmetic blemish occurs. A sporadic supernumerary nipple typically does not present an indication for a thorough workup for other malformations (see Consultations).

Author

Aryeh Metzker, MD, Consulting Staff, Department of Pediatric Dermatology, Senior Clinical Lecturer, Department of Dermatology, Sourasky Medical Center, Sackler School of Medicine, Tel Aviv University

Disclosure: Nothing to disclose.

Specialty Editors

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School

Disclosure: Nothing to disclose.

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Disclosure: Abvie Honoraria Speaking and teaching

Chief Editor

Dirk M Elston, MD, Director, Ackerman Academy of Dermatopathology, New York

Disclosure: Nothing to disclose.

Additional Contributors

Mark A Crowe, MD Assistant Clinical Instructor, Department of Medicine, Division of Dermatology, University of Washington School of Medicine

Mark A Crowe, MD is a member of the following medical societies: American Academy of Dermatology and North American Clinical Dermatologic Society

Disclosure: Nothing to disclose.

References

  1. Conde DM, Kashimoto E, Torresan RZ, Alvarenga M. Pseudomamma on the foot: an unusual presentation of supernumerary breast tissue. Dermatol Online J. May 30 2006;12(4):7. [View Abstract]
  2. De Cholnoky T. Supernumerary breast. Arch Surg. 1939;39:926-41.
  3. Grossl NA. Supernumerary breast tissue: historical perspectives and clinical features. South Med J. Jan 2000;93(1):29-32. [View Abstract]
  4. Leichtenstern D. Uber das Vorkommen und Bedeutung Supernumerarer (accessorischer ) Bruste und Brustwarzen. Arch Pathol Anat Physiol Klin Med. 1878;73:222.
  5. Aslan D, Gürsel T, Kaya Z. Supernumerary nipples in children with hematologic disorders. Pediatr Hematol Oncol. Jul-Aug 2004;21(5):461-3. [View Abstract]
  6. Goldschmidt E, Jacobsen N. Epibulbar lipodermoids, preauricular appendages and polythelia in four generations: a new hereditary syndrome?. Ophthalmic Genet. Jun 2010;31(2):81-3. [View Abstract]
  7. Méhes K. Association of supernumerary nipples with other anomalies. J Pediatr. Aug 1979;95(2):274-5. [View Abstract]
  8. Mehes K. Association of supernumerary nipples with other anomalies. J Pediatr. Jan 1983;102(1):161. [View Abstract]
  9. Varsano IB, Jaber L, Garty BZ, Mukamel MM, Grünebaum M. Urinary tract abnormalities in children with supernumerary nipples. Pediatrics. Jan 1984;73(1):103-5. [View Abstract]
  10. Goedert JJ, McKeen EA, Fraumeni JF Jr. Polymastia and renal adenocarcinoma. Ann Intern Med. Aug 1981;95(2):182-4. [View Abstract]
  11. Kahn SA, Wagner RF Jr. Polythelia and unilateral renal agenesis. Cutis. Aug 1982;30(2):225-6. [View Abstract]
  12. Meggyessy V, Mehes K. Association of supernumerary nipples with renal anomalies. J Pediatr. Sep 1987;111(3):412-3. [View Abstract]
  13. Hersh JH, Bloom AS, Cromer AO, Harrison HL, Weisskopf B. Does a supernumerary nipple/renal field defect exist?. Am J Dis Child. Sep 1987;141(9):989-91. [View Abstract]
  14. Mehes K, Pinter A. Minor morphological aberrations in children with isolated urinary tract malformations. Eur J Pediatr. Mar 1990;149(6):399-402. [View Abstract]
  15. Leung AK, Robson WL. Renal anomalies in familial polythelia. Am J Dis Child. Jun 1990;144(6):619-20. [View Abstract]
  16. Urbani CE, Betti R. Accessory mammary tissue associated with congenital and hereditary nephrourinary malformations. Int J Dermatol. May 1996;35(5):349-52. [View Abstract]
  17. Brown J, Schwartz RA. Supernumerary nipples and renal malformations: a family study. J Cutan Med Surg. May-Jun 2004;8(3):170-2. [View Abstract]
  18. Ferrara P, Giorgio V, Vitelli O, Gatto A, Romano V, Bufalo FD, et al. Polythelia still a marker of urinary trct anomalies in children. Scand J Urol Nephrol. 2009;43(1):47-50.
  19. Grimshaw EC, Cohen PR. Supernumerary nipple and seminoma: case report and review of polythelia and genitourinary cancers. Dermatol Online J. Jan 15 2013;19(1):4. [View Abstract]
  20. Smith DW. Comment on association of supernumerary nipples with other anomalies. In: Oska FA, Stockman JA III, eds. Year Book of Pediatrics. Chicago, Ill: YB Medical Publishers; 1981:437.
  21. Rahbar F. Clinical significance of supernumerary nipples in black neonates. Clin Pediatr (Phila). Jan 1982;21(1):46-7. [View Abstract]
  22. Mimouni F, Merlob P, Reisner SH. Occurrence of supernumerary nipples in newborns. Am J Dis Child. Oct 1983;137(10):952-3. [View Abstract]
  23. Robertson A, Sale P, Sathyanarayan. Lack of association of supernumerary nipples with renal anomalies in black infants. J Pediatr. Sep 1986;109(3):502-3. [View Abstract]
  24. Kenney RD, Flippo JL, Black EB. Supernumerary nipples and renal anomalies in neonates. Am J Dis Child. Sep 1987;141(9):987-8. [View Abstract]
  25. Hoyme HE. Minor malformations. Significant or insignificant?. Am J Dis Child. Sep 1987;141(9):947. [View Abstract]
  26. Bortz J, Parker S, Ray TL. Lack of associated renal anomalies in familial polythelia. Am J Dis Child. Aug 1989;143(8):883. [View Abstract]
  27. Armoni M, Filk D, Schlesinger M, Pollak S, Metzker A. Accessory nipples: any relationship to urinary tract malformation?. Pediatr Dermatol. Sep 1992;9(3):239-40. [View Abstract]
  28. Jojart G, Seres E. Supernumerary nipples and renal anomalies. Int Urol Nephrol. 1994;26(2):141-4. [View Abstract]
  29. Casey HD, Chasan PE, Chick LR. Familial polythelia without associated anomalies. Ann Plast Surg. Jan 1996;36(1):101-4. [View Abstract]
  30. Schmidt H. Supernumerary nipples: prevalence, size, sex and side predilection -- a prospective clinical study. Eur J Pediatr. Oct 1998;157(10):821-3. [View Abstract]
  31. Grotto I, Browner-Elhanan K, Mimouni D, Varsano I, Cohen HA, Mimouni M. Occurrence of supernumerary nipples in children with kidney and urinary tract malformations. Pediatr Dermatol. Jul-Aug 2001;18(4):291-4. [View Abstract]
  32. Newman M. Supernumerary nipples. Am Fam Physician. Aug 1988;38(2):183-8. [View Abstract]
  33. Baruchin A, Rosenberg L. A supernumerary nipple in a rare site. Report of a case. J Dermatol Surg Oncol. Nov 1981;7(11):918-9. [View Abstract]
  34. Hanson E, Segovia J. Dorsal supernumerary breast. Case report. Plast Reconstr Surg. Mar 1978;61(3):441-5. [View Abstract]
  35. Jaber L, Merlob P. The prevalence of supernumerary nipples in Arab infants and children. Eur J Pediatr. May 1988;147(4):443. [View Abstract]
  36. Oztas MO, Gurer MA. Dermoscopic features of accessory nipples. Int J Dermatol. Oct 2007;46(10):1067-8. [View Abstract]
  37. Kanitakis J. In vivo reflectance confocal microscopy of supernumerary nipple and differential diagnosis from melanocytic lesions. J Cutan Pathol. Jul 2010;37(7):797-801. [View Abstract]
  38. Schewach-Millet M, Fisher BK. Supernumerary nipple on the shoulder. Cutis. Feb 1976;17(2):384-5. [View Abstract]
  39. Toumbis-Ioannou E, Cohen PR. Familial polythelia. J Am Acad Dermatol. Apr 1994;30(4):667-8. [View Abstract]
  40. Shewmake SW, Izuno GT. Supernumerary areolae. Arch Dermatol. Jun 1977;113(6):823-5. [View Abstract]
  41. Willman JH, Golitz LE, Fitzpatrick JE. Clear cells of Toker in accessory nipples. J Cutan Pathol. Apr 2003;30(4):256-60. [View Abstract]
  42. Sladden MJ, Sladden JA. A novel treatment for accessory nipples. Br J Dermatol. Feb 2008;158(2):405. [View Abstract]
  43. Madej B, Balak B, Winkler I, Burdan F. Cancer of the accessory breast--a case report. Adv Med Sci. 2009;54(2):308-10. [View Abstract]
  44. Fan J. Removal of accessory breasts: a novel tumescent liposuction approach. Aesthetic Plast Surg. Nov 2009;33(6):809-13. [View Abstract]
  45. Cinpolat A, Bektas G, Seyhan T, Ozad U, Coskunfirat OK. Treatment of a supernumerary large breast with medial pedicle reduction mammaplasty. Aesthetic Plast Surg. Aug 2013;37(4):762-6. [View Abstract]

Supernumerary nipple in a neonate. Courtesy of Dr P. Merlob.

Supernumerary nipple in an adolescent boy. Courtesy of Dr B. Fisher.

Supernumerary nipple (bilateral) in an adolescent girl.

Ectopic supernumerary nipple on the shoulder. Courtesy of Dr B. Fisher.

Supernumerary nipple in a neonate. Courtesy of Dr P. Merlob.

Supernumerary nipple (bilateral) in an adolescent girl.

Supernumerary nipple in an adolescent boy. Courtesy of Dr B. Fisher.

Ectopic supernumerary nipple on the shoulder. Courtesy of Dr B. Fisher.