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Hyperkeratosis of the Nipple and Areola

Background

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
:

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.
Others have advocated that the term “nevoid” be replaced by “idiopathic”.
Upon review of the literature, a recommended classification is into (1) primary hyperkeratosis of the nipple and/or areola, which is idiopathic,
and (2) secondary hyperkeratosis of the nipple and/or areola, which is associated with the following:

Epidermal nevus

Organoid nevus

Leiomyomas

Verruca

Congenital, acquired, or erythrodermic ichthyosis

Malignant acanthosis nigricans

Darier disease

Chronic eczema such as atopic dermatitis

Cutaneous T-cell lymphoma

Chronic mucocutaneous candidiasis

Pregnant females

Males receiving hormonal therapy for prostate cancer

Estrogen therapy for androgen insensitivity syndrome

Vemurafenib treatment

Malassezia furfur infection

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