Hyperkeratosis of the Nipple and Areola

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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]

Darier disease^[16]

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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