Hyperkeratosis of the Nipple and Areola Clinical Presentation

  • Author: Joseph C English III, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 20, 2010
 

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[12, 13, 22, 23] 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 guidelines for early detection of breast cancer.[24] 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:

  • Begin mammography at age 40 years.
  • For women in their 20s and 30s, it is recommended that clinical breast examination be part of a periodic health examination, preferably at least every 3 years. Asymptomatic women aged 40 years and older should continue to receive a clinical breast examination as part of a periodic health examination, preferably annually.
  • Beginning in their 20s, women should be told about the benefits and limitations of breast self-examinations (BSEs). The importance of prompt reporting of any new breast symptoms to a health professional should be emphasized. Women who choose to do BSE should receive instruction and should have their technique reviewed on the occasion of a periodic health examination. It is acceptable for women to choose not to do a BSE or to do BSE irregularly.
  • Women should have the opportunity to become informed about the benefits, limitations, and potential harms associated with regular screening.
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Physical

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 hyperkeratosiAn adult male with bilateral primary hyperkeratosis of the nipple and areola A close-up of the patient's right nipple/areola coA 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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Contributor Information and Disclosures
Author

Joseph C English III, MD  Clinical Vice-Chairman for Quality and Innovation, Associate Professor of Dermatology, Department of Dermatology, University of Pittsburgh

Joseph C English III, MD is a member of the following medical societies: American Academy of Dermatology and American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Kathryn Schwarzenberger, MD  Associate Professor of Medicine, Division of Dermatology, University of Vermont College of Medicine; Consulting Staff, Division of Dermatology, Fletcher Allen Health Care

Kathryn Schwarzenberger, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, American Dermatological Association, Dermatology Foundation, Medical Dermatology Society, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

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

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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