eMedicine Specialties > Dermatology > Papulosquamous Diseases

Hyperkeratosis of the Nipple and Areola

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

Updated: Sep 19, 2008

Introduction

Background

Hyperkeratosis of the nipple and/or areola (HNA) is defined as excessive keratinization of the nipple and/or areola. HNA is characterized by hyperpigmented, verrucous or filiform, keratotic thickening of the nipple and/or areola, with a papillomatosis or velvety sensation to touch.

HNA is classified into 3 subsets, as follows:

This discussion focuses on type III, or nevoid, HNA. The use of the term nevoid has been questioned by some authors. They prefer type III, or idiopathic, HNA. Because modern textbooks and articles to date continue to use the term nevoid, it remains in use for this article.

The Medscape Breast Cancer Resource Center may be of interest.

Frequency

United States

Type III or nevoid HNA is rare.

International

Type III or nevoid HNA is rare.

Mortality/Morbidity

Mortality is not associated with nevoid HNA. The morbidity rate is low, and morbidity is primarily limited to the undesirable cosmetic results of the abnormal nipple and/or areola. The morbidity and mortality rates of type II HNA are those of the underlying diseases; thus, the rates with type II HNA may be greater than those with other types of HNA.

Race

Nevoid HNA has no reported racial predilection.

Sex

Nevoid HNA is more common in females than in males.

Age

In females, nevoid HNA most commonly occurs in those aged 10-40 years. Males with nevoid HNA are often older than females, but no specific age distribution is reported.

Clinical

History

  • The diagnosis of type I hyperkeratosis of the nipple and areola (HNA) and type II HNA are usually straightforward because the other cutaneous manifestations of the associated skin diseases are apparent.
    • Type I HNA associated with an epidermal nevus may involve only 1 nipple and/or areola.
    • Type II HNA is frequently bilateral; this feature reflects the more widespread or systemic nature of the underlying disease.
  • Type III, or nevoid HNA, is not associated with any underlying conditions and is an isolated finding. Skin biopsy findings may confirm the diagnosis if it is unclear from the clinical presentation.
    • Nevoid HNA can occur unilaterally, but it most frequently affects both sides. In greater than 50% of the cases, nevoid HNA affects both the nipple and areola.
    • Women with unilateral lesions may have bilateral disease during pregnancy.
    • Pregnancy may also produce thicker, darker lesions.
  • The cutaneous changes of nevoid HNA 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 HNA5 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.
    • 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 is also indicated.
  • The American Cancer Society has guidelines for the detection of breast cancer in asymptomatic women.6 These guidelines apply to women with the usual risk for breast cancer and no symptoms of breast cancer. Women with certain risk factors, such as a family history of breast cancer, should discuss their risk factors with their doctor. The guidelines are the following:
    • Breast self-examination (BSE) is an option for women starting in their 20s. Women should be told about the benefits and limitations of BSE. Women should immediately report any breast changes to their health care professional.
    • Women in their 20s and 30s should have a clinical breast examination (CBE) as part of a periodic (regular) health examination by a health care professional, preferably every 3 years. After age 40 years, women should have a CBE by a health professional every year.
    • Women aged 40 years and older should undergo mammography every year and should continue to do so as long as they are in good health.
    • Women at high risk (greater than 20% lifetime risk) should have an MRI and a mammogram every year. Women of moderately increased risk (15-20% lifetime risk) should talk with their doctors about the benefits and limitations of adding MRI screening to their yearly mammogram. Yearly MRI screening is not recommended for women whose lifetime risk of breast cancer is less than 15%. Please see the American Cancer Society Web site for more specifics concerning lifetime risk assessment for breast cancer.

Physical

  • The affected nipple and/or areola are thickened and may be covered with hyperpigmented verrucous or filiform hyperkeratotic plaques (see Media File 1).
  • No associated abnormalities of nipple and/or areola anatomy and underlying breast should be present.

Causes

  • The etiology of nevoid HNA is unknown.
  • Some authors believe that hormonal causes are involved because the condition may occur or change at puberty, during pregnancy, or during hormonal therapy for prostate cancer.7
  • Many cases are idiopathic.

More on Hyperkeratosis of the Nipple and Areola

Overview: Hyperkeratosis of the Nipple and Areola
Differential Diagnoses & Workup: Hyperkeratosis of the Nipple and Areola
Treatment & Medication: Hyperkeratosis of the Nipple and Areola
Follow-up: Hyperkeratosis of the Nipple and Areola
Multimedia: Hyperkeratosis of the Nipple and Areola
References
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References

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  2. Fitzgerald DA, Lewis-Jones MS. Darier's disease presenting as isolated hyperkeratosis of the breasts. Br J Dermatol. Feb 1997;136(2):290. [Medline].

  3. Ahn SK, Chung J, Soo Lee W, Kim SC, Lee SH. Hyperkeratosis of the nipple and areola simultaneously developing with cutaneous T-cell lymphoma. J Am Acad Dermatol. Jan 1995;32(1):124-5. [Medline].

  4. Allegue F, Soria C, Rocamora A, Fraile G, Ledo A. Hyperkeratosis of the nipple and areola in a patient with cutaneous T-cell lymphoma. Int J Dermatol. Sep 1990;29(7):519-20. [Medline].

  5. D'Souza M, Gharami R, Ratnakar C, Garg BR. Unilateral nevoid hyperkeratosis of the nipple and areola. Int J Dermatol. Aug 1996;35(8):602-3. [Medline].

  6. Leitch AM, Dodd GD, Costanza M, Linver M, Pressman P, McGinnis L, et al. American Cancer Society guidelines for the early detection of breast cancer: update 1997. CA Cancer J Clin. May-Jun 1997;47(3):150-3. [Medline].

  7. Lambiris AG, McCormick F. Unilateral hyperkeratosis of nipple and areola associated with androgen insensitivity and oestrogen replacement therapy. J Eur Acad Dermatol Venereol. Jul 2001;15(4):376-7. [Medline].

  8. English JC 3rd, Coots NV. A man with nevoid hyperkeratosis of the areola. Cutis. May 1996;57(5):354-6. [Medline].

  9. Okan G, Baykal C. Nevoid hyperkeratosis of the nipple and areola: treatment with topical retinoic acid. J Eur Acad Dermatol Venereol. Nov 1999;13(3):218-20. [Medline].

  10. Pérez-Izquierdo JM, Vilata JJ, Sánchez JL, Gargallo E, Millan F, Aliaga A. Retinoic acid treatment of nipple hyperkeratosis. Arch Dermatol. May 1990;126(5):687-8. [Medline].

  11. Bayramgürler D, Bilen N, Apaydin R, Erçin C. Nevoid hyperkeratosis of the nipple and areola: treatment of two patients with topical calcipotriol. J Am Acad Dermatol. Jan 2002;46(1):131-3. [Medline].

  12. Durmazlar SP, Eskioglu F, Bodur Z. Hyperkeratosis of the nipple and areola: 2 years of remission with low-dose acitretin and topical calcipotriol therapy. J Dermatolog Treat. May 30 2008;1-4. [Medline].

  13. Lee HW, Lee MW, Choi JH, Moon KC, Koh JK. To the editor: Unilateral nevoid hyperkeratosis of the nipple and areola: excellent response to cryotherapy. Dermatol Surg. May 2005;31(5):611-2. [Medline].

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  16. Milanovic R, Martic K, Stanec S, Zic R, Vlajcic Z, Stanec Z. Surgical treatment of nevoid hyperkeratosis of the areola by removal of the areola and reconstruction with a skin graft. Ann Plast Surg. Jun 2005;54(6):667-9. [Medline].

  17. Busse A, Peschen M, Schöpf E, Vanscheidt W. Treatment of hyperkeratosis areolae mammae naeviformis with the carbon dioxide laser. J Am Acad Dermatol. Aug 1999;41(2 Pt 1):274-6. [Medline].

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  19. Alpsoy E, Yilmaz E, Aykol A. Hyperkeratosis of the nipple: report of two cases. J Dermatol. Jan 1997;24(1):43-5. [Medline].

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  21. Baykal C, Büyükbabani N, Kavak A, Alper M. Nevoid hyperkeratosis of the nipple and areola: a distinct entity. J Am Acad Dermatol. Mar 2002;46(3):414-8. [Medline].

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  23. Krishnan RS, Angel TA, Roark TR, Hsu S. Nevoid hyperkeratosis of the nipple and/or areola: a report of two cases and a review of the literature. Int J Dermatol. Nov 2002;41(11):775-7. [Medline].

  24. Kubota Y, Koga T, Nakayama J, Kiryu H. Naevoid hyperkeratosis of the nipple and areola in a man. Br J Dermatol. Feb 2000;142(2):382-4. [Medline].

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  31. Ratón JA, Bilbao I, Gardeazábal J, Alvarez S, Vicente JM, Gonzalez R, et al. Skin involvement in male breast carcinoma. Arch Dermatol. Apr 1998;134(4):517-8. [Medline].

  32. Revert A, Bañuls J, Montesinos E, Jorda E, Ramon D, Torres V. Nevoid hyperkeratosis of the areola. Int J Dermatol. Oct 1993;32(10):745-6. [Medline].

  33. Schwartz RA. Hyperkeratosis of nipple and areola. Arch Dermatol. Dec 1978;114(12):1844-5. [Medline].

  34. Sengül N, Parlak AH, Oruk S, Boran C. Nevoid hyperkeratosis of the nipple and areola: a diagnosis of exclusion. Breast J. Jul-Aug 2006;12(4):383-4. [Medline].

  35. Shastry V, Betkerur J, Kushalappa PA. Unilateral nevoid hyperkeratosis of the nipple: a report of two cases. Indian J Dermatol Venereol Leprol. Jul-Aug 2006;72(4):303-5. [Medline].

  36. Soden CE. Hyperkeratosis of the nipple and areola. Cutis. Jul 1983;32(1):69-71, 74. [Medline].

  37. Trattner A, David M, Sandbank M. Seborrheic keratoses of the areola. Cutis. Feb 1994;53(2):95-6. [Medline].

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Further Reading

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Keywords

nipple, areola, HNA, HNA type 1, HNA type 2, HNA type 3, nevoid HNA, excessive keratinization of the nipple and/or areola

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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.

Medical Editor

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.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
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.

Managing Editor

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.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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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