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AUTHOR AND EDITOR INFORMATION

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Author: Joseph C English III, MD, Residency Program Director, Associate Professor of Dermatology, Department of Dermatology, University of Pittsburgh

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

Editors: 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; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center

Author and Editor Disclosure

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

INTRODUCTION

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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:

  • Type I - HNA due to the extension of a epidermal nevus
  • Type II - HNA in conjunction with disseminated dermatoses
    • Congenital, acquired, or erythrodermic ichthyosis
    • Acanthosis nigricans
    • Darier disease
    • Chronic eczema such as atopic dermatitis
    • Cutaneous T-cell lymphoma
  • Type III - Nevoid HNA
    • Females in puberty, those of childbearing age, pregnant females
    • Males receiving hormonal (castration) therapy for prostate cancer
    • Males and females without the above conditions

This discussion focuses on type III, or nevoid, HNA.

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

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History

  • The diagnosis of type I 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 HNA 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. 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:
    • Women aged 20 years and older should perform breast self-examination every month.
    • In women aged 20-39 years a health care professional, such as a physician, physician assistant, nurse, or nurse practitioner, should physically examine the breasts every 3 years.
    • Women aged 40 years and older should undergo mammography and should have their breasts examined by a health care professional every year.

Physical

  • The affected nipple and/or areola are thickened and may be covered with hyperpigmented verrucous or filiform hyperkeratotic plaques (see Image 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.
  • Many cases are idiopathic.


DIFFERENTIALS

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Fox-Fordyce Disease
Seborrheic Keratosis

Other Problems to be Considered

Chronic, dry nipple eczema
Jogger's nipple, or friction or irritant-induced dermatitis with lichenification
Localized Darier disease of the breast


WORKUP

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Lab Studies

  • No blood or urine laboratory tests aid in the diagnosis of nevoid HNA.

Imaging Studies

  • Imaging studies must be performed if any concern about underlying breast disease exists.
  • Mammography should be performed, with or without ultrasonography.

Other Tests

  • The acquisition of a skin biopsy sample for histopathologic examination aids in differentiating nevoid HNA from other conditions.
  • The preferred method involves a 3- or 4-mm punch biopsy followed by closure with 6-0 Prolene sutures.
  • An alternative suture material, 5-0 or 6-0 plain gut (absorbable) suture may be used if desired.
  • This method provides the best cosmetic results, with minimal scarring and maintenance of the normal architecture of the nipple and/or areola.

Histologic Findings

Nevoid HNA is characterized by variable orthokeratotic hyperkeratosis, slight acanthosis, and marked papillomatosis changes on routine hematoxylin-eosin–stained specimens. In type II HNA, biopsy samples may reveal histologic findings related to the associated skin disease.


TREATMENT

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Medical Care

  • The clinical course of nevoid HNA is variable and often unpredictable.
  • Many treatment modalities for nevoid HNA have been used; however, the results of most are anecdotal and not verified in randomized, placebo-controlled clinical trials.
  • In addition, reported results vary among individual patients and with various medications.
  • Reported treatments of nevoid HNA include the following:
    • Lactic acid 12% cream
    • Salicylic acid gel 6%
    • Topical corticosteroids
    • Topical tretinoin
    • Topical calcipotriol
    • Cryotherapy
    • Shave or surgical excision of involved portion
    • Surgical areola removal and skin graft reconstruction
    • Carbon dioxide laser removal
    • Radiofrequency surgery
  • Therapeutic options for type I HNA and type II HNA consist of the treatment options for the underlying dermatologic condition.

Consultations

In some cases, consultation with a plastic surgeon may be appropriate.


FOLLOW-UP

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Further Outpatient Care

  • The follow-up care for patients with nevoid HNA is based on the response to therapy and any change in the clinical presentation.
  • A follow-up examination at 3-6 months after the initiation of therapy is reasonable.
  • The patient should be instructed to return to the clinic immediately if any nipple discharge, nipple retraction, or palpable mass is present.
  • Monthly breast examinations are important for breast cancer surveillance, and they are mandatory for all patients with nevoid HNA, especially if the disease is unilateral.
  • Patients should be cautioned not to attribute any changes in their breasts to the associated HNA.

Patient Education

  • Patient education of nevoid HNA is important to help individuals understand their condition and to allow them to form realistic expectations regarding treatment.
  • Patients should be warned that, with topical preparations, a long period may pass before clinical improvement occurs.
  • Treatment with an individual medication should be continued for at least 6 months before it is deemed a failure.
  • Lesions may recur after therapy is discontinued.
  • In some patients, HNA does not respond to any treatment.


MISCELLANEOUS

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Medical/Legal Pitfalls

  • Failure to diagnose a more serious underlying condition might be the only downfall for the dermatologist.
  • Documenting features of the patient's history and findings from the physical examination is important.
  • Any aberration should be further evaluated to rule out a more serious process (eg, breast carcinoma), as reviewed previously.


MULTIMEDIA

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Media file 1:  Hyperkeratosis of the areola.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo


REFERENCES

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Hyperkeratosis of the Nipple and Areola excerpt

Article Last Updated: Sep 1, 2006