Supernumerary nipples, also known as accessory nipples or polythelia, are a common congenital malformation. The nipples may be either along the embryonic milk lines or beyond the milk lines, the latter type are called ectopic supernumerary nipples. Much more rarely, the nipples appear with complete breast tissue and ducts, this is known as polymastia.
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Epidemiology
Associations
Supernumerary nipples may be found in association with many syndromes and other conditions:
Syndromes
Central nervous system
neurosis
familial alcoholism
developmental delay
Gastrointestinal
Ear, nose, throat and lung
ear abnormalities
Skeletal
hand malformation
absent rib
coronal suture synostosis
Cardiac
conduction defect
bundle branch block
Clinical presentation
May remain undetected. Occasionally, the supernumerary nipple is noticed when hormonal changes during adolescence, menstruation, or pregnancy cause increased pigmentation, fluctuating swelling, tenderness, or even lactation.
Pathology
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).
Treatment and prognosis
Usually no treatment is required; however, a protruding embarrassing supernumerary nipple can be removed surgically, if desired. Removal using liquid nitrogen cryotherapy has also been described.
Differential diagnosis
pigmented nevus or other skin lesions
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