Acanthosis nigricans may have a similar histological aspect as hyperkeratosis of the nipple and areola however, it involves mainly flexural areas and is often associated with metabolic disorders. Furthermore, in seborrheic keratosis, a discrete fibrosis of the upper dermis is usually observed on histological examination. Seborrheic keratosis shares similar histological features with hyperkeratosis of the nipple and areola, and the diagnosis of seborrheic keratosis is likely if the papillomatous papules are well delimited. The diagnosis is all the more difficult as the histological aspects are quite similar, such as in seborrheic keratosis, acanthosis nigricans, and epidermal nevus. It may be challenging to differentiate the idiopathic type of hyperkeratosis of the nipple and areola from secondary type. In pregnant women, some physiological changes of the breast (such as pigmentation, and nipple and areola enlargement) should be considered in the differential diagnosis of hyperkeratosis of the nipple and areola. This second type of hyperkeratosis of the nipple and areola also includes breast lesions developed in pregnant women and males receiving estrogen therapy.
The classification initially proposed by Levy-Franckel divides hyperkeratosis of the nipple and areola into three types: (1) epidermal nevus, with unilateral involvement of a single breast, usually in a Blaschko linear pattern (observed equally in both genders) (2) hyperkeratosis of the nipple and areola associated with ichthyosis, with bilateral involvement of the breasts (observed equally in both genders) and (3) a bilateral type, generally affecting both nipples and areolae and observed mainly in females in the second or third decade of life.Ī more recent classification divides hyperkeratosis of the nipple and areola into two types: (1) a primary or idiopathic type, and (2) a secondary type, associated with another skin condition such a naevi structure (mainly epidermal nevus but also organoid nevus leiomyomas) or a warty tumor (verruca, seborrheic keratosis), as well as ichthyosis, chronic eczema (including atopic dermatitis), Darier disease, acanthosis nigricans, terra firma dermatitis, reticulate and confluent papillomatosis, and cutaneous T-cell lymphoma. No prepubertal onset of hyperkeratosis of the nipple and areola has been observed. In some women, breastfeeding may become impossible because of nipple lesions. The skin thickening is variable from a simple hyperkeratosis to a tumoral aspect. Skin lesions may become darker and thicker during pregnancy and tend to improve after delivery. There is usually a diffuse pigmentation and a warty thickening of the nipples and/or areolae. Hyperkeratosis of the nipple and areola occurs during puberty or pregnancy. In males under estrogen therapy, a gynecomastia may be observed in association with hyperkeratosis of the nipple and areola. However, pruritus may be present in some patients. The skin lesions are usually painless with no discharge or associated lymphadenopathy. Rarely, hyperkeratotic patches may extend beyond the areolar region. It may affect only the nipple, areola or both. Hyperkeratosis of the nipple and areola may be unilateral or bilateral. This activity reviews the etiology, presentation, evaluation, and management of hyperkeratosis of the nipple and areola and reviews the role of the interprofessional team in evaluating, diagnosing, and managing the condition. Physical destructive treatments, such as cryotherapy with liquid nitrogen, and carbon dioxide laser give more satisfactory results. Systemic retinoids (etretinate, acitretin) were inefficient when used. Topical corticosteroids and retinoids have almost no effect, even if they may relieve pruritus. Nevertheless, they are rarely sufficient. Keratolytic topicals (salicylic acid, lactic acid) are used as a first-line treatment. Various medical or surgical treatments may be indicated in this condition. Hyperkeratosis of the nipple and areola does not resolve without treatment and tends to persist indefinitely. This activity describes the pathophysiology and presentation of nipple hyperkeratosis and its treatment. Hyperkeratosis of the nipple and areola is an uncommon, benign skin condition characterized by a warty thickening and pigmentation of the nipple, areola, or both.