Hyperkeratosis of the Nipple and Areola
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Hyperkeratosis of the nipple and/or areola (HNA) is defined as excessive keratinization of the nipple and/or areola. Hyperkeratosis of the nipple and/or areola is characterized by hyperpigmented, verrucous or filiform, keratotic thickening of the nipple and/or areola, with a papillomatosis or velvety sensation to touch. Hyperkeratosis of the nipple and/or areola has classically been classified into the following three subsets [1, 2, 3] :
Type I – hyperkeratosis of the nipple and/or areola due to the extension of an epidermal nevus
Type II – hyperkeratosis of the nipple and/or areola in conjunction with disseminated dermatoses
Type III – Nevoid hyperkeratosis of the nipple and/or areola
Perez-Izquierdo et al suggested an alternative classification, distinguishing two types: (1) those that are idiopathic or nevoid and (2) those that are secondary to other conditions. [4] Others have advocated that the term “nevoid” be replaced by “idiopathic”. [5, 6] Upon review of the literature, a recommended classification is into (1) primary hyperkeratosis of the nipple and/or areola, which is idiopathic, [7, 8, 9, 10, 11] and (2) secondary hyperkeratosis of the nipple and/or areola, which is associated with the following:
Epidermal nevus
Organoid nevus [12]
Leiomyomas [13]
Verruca [14]
Congenital, acquired, or erythrodermic ichthyosis
Malignant acanthosis nigricans [15]
Chronic eczema such as atopic dermatitis
Cutaneous T-cell lymphoma [17, 18]
Chronic mucocutaneous candidiasis [19]
Pregnant females
Males receiving hormonal therapy for prostate cancer [20, 21]
Estrogen therapy for androgen insensitivity syndrome [22]
Vemurafenib treatment [23]
Malassezia furfur infection [24]
The pathophysiology of hyperkeratosis of the nipple and/or areola is unknown.
Hyperkeratosis of the nipple and/or areola is rare, and currently no domestic or international incidence rates have been documented in the literature.
Hyperkeratosis of the nipple and/or areola has no reported racial predilection.
Hyperkeratosis of the nipple and/or areola is more common in females than in males. In a study of 45 primary hyperkeratosis of the nipple and/or areola patients 80% were females. [25]
In females, hyperkeratosis of the nipple and/or areola most commonly occurs in those aged 10-40 years. Males with nevoid hyperkeratosis of the nipple and/or areola are often older than females, but no specific age distribution is reported.
Primary hyperkeratosis of the nipple and/or areola is not associated with mortality. 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 secondary hyperkeratosis of the nipple and/or areola are those of the underlying diseases; thus, the rates with secondary hyperkeratosis of the nipple and/or areola may be greater than those with other types of hyperkeratosis of the nipple and/or areola.
Patient education of nevoid hyperkeratosis of the nipple and/or areola 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, hyperkeratosis of the nipple and/or areola does not respond to any treatment.
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Rabindranath Nambi, MD Consulting Staff, Department of Dermatology, Dudley Group of Hospitals, UK
Disclosure: Nothing to disclose.
Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology
Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, 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, Society for Investigative Dermatology
Disclosure: Nothing to disclose.
Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.
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: Women’s Dermatologic Society, American Contact Dermatitis Society, Medical Dermatology Society, Dermatology Foundation, Alpha Omega Alpha, American Academy of Dermatology
Disclosure: Nothing to disclose.
Ivan D Camacho, MD Dermatologist, Private Practice; Voluntary Assistant Professor of Dermatology, Department of Dermatology and Cutaneous Surgery, University of Miami, Leonard M Miller School of Medicine
Ivan D Camacho, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Florida Medical Association, International Society of Dermatology, Women’s Dermatologic Society
Disclosure: Nothing to disclose.
Joshua R Freedman, MD, MS Resident Physician, Department of Dermatology and Cutaneous Surgery, Jackson Memorial Hospital, University of Miami, Leonard M Miller School of Medicine
Joshua R Freedman, MD, MS is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.
Joseph C English III, MD Clinical Vice-Chairman for Quality and Innovation, Professor of Dermatology, Department of Dermatology, University of Pittsburgh School of Medicine
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.
Hyperkeratosis of the Nipple and Areola
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