Hyperkeratosis of the Nipple and Areola Clinical Presentation

Updated: May 02, 2017
  • Author: Rabindranath Nambi, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

The diagnosis of secondary hyperkeratosis of the nipple and/or areola is usually straightforward because the other cutaneous manifestations of the associated skin diseases that are the cause of the disease are apparent. Secondary hyperkeratosis of the nipple and/or areola associated with an epidermal nevus, organoid nevus, leiomyoma, or verruca usually involves only 1 nipple and/or areola. Secondary hyperkeratosis of the nipple and/or areola is bilateral when this clinical manifestation reflects the more widespread or systemic nature of the underlying disease. Women with unilateral primary hyperkeratosis of the nipple and/or areola lesions may have bilateral disease during pregnancy (secondary hyperkeratosis of the nipple and/or areola) Pregnancy may also produce thicker, darker lesions.

Primary hyperkeratosis of the nipple and/or areola is not associated with any underlying conditions and is an isolated finding. Skin biopsy findings may help confirm the diagnosis if it is unclear from the clinical presentation. Primary hyperkeratosis of the nipple and/or areola can occur unilaterally, but it most frequently affects both sides. In greater than 50% of the cases, primary hyperkeratosis of the nipple and/or areola affects both the nipple and areola. The cutaneous changes of nevoid hyperkeratosis of the nipple and/or areola are generally asymptomatic. Mild pruritus may result from poor hygiene. Most complaints reflect concern about the cosmetic appearance of the thickened hyperpigmented nipples and/or areolas.

Unilateral primary hyperkeratosis of the nipple and/or areola [14, 15, 26, 27] must be distinguished from an underlying breast carcinoma. Pain, bleeding, ulceration, a nipple discharge, or loss of normal anatomy with nipple retraction or loss of nipple should prompt immediate evaluation. Lesions recalcitrant to therapy also warrant investigation (ie, biopsy). The evaluation of these lesions should include complete bilateral breast examination with evaluation of the lymph nodes, mammography, and biopsy of the involved skin. Consultation with a surgical oncologist may be indicated if any of the above occurs.

The American Cancer Society has released 2012 guidelines for early detection of breast cancer. [28] These guidelines apply to women with the usual (average) risk for breast cancer and no symptoms of breast cancer. Older women or high-risk women (ie, family history of breast cancer) should discuss their risk factors with their doctor for more in-depth guidelines. For average-risk women, the guidelines are as follows:

  • Early mammograms are recommended starting at age 40 years and continuing for as long as a woman is in good health.

  • Clinical breast examination (CBE) should be performed about every 3 years for women in their 20s and 30s and every year for women aged 40 years and older.

  • Women should know how their breasts normally look and feel and report any breast change promptly to their healthcare provider. Breast self-examination (BSE) is an option for women starting in their 20s.

  • Some womenm because of their family history, a genetic tendency, or certain other factors, should be screened with MRI in addition to mammography.

  • Women should have the opportunity to become informed about the benefits, limitations, and potential harms associated with regular screening.

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Physical Examination

The affected nipple and/or areola are thickened and may be covered with hyperpigmented verrucous or filiform hyperkeratotic plaques (see the images below).

An adult male with bilateral primary hyperkeratosi An adult male with bilateral primary hyperkeratosis of the nipple and areola
A close-up of the patient's right nipple/areola co A close-up of the patient's right nipple/areola complex.
A close up of the patient's nipple/areola complex. A close up of the patient's nipple/areola complex.

No associated abnormalities of nipple and/or areola anatomy and underlying breast should be present.

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Causes

The etiology of primary hyperkeratosis of the nipple and/or areola is unknown. The etiology of secondary hyperkeratosis of the nipple and/or areola is related to the underlying disorder.

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