Nevoid hyperkeratosis of the nipple and the areola

Nevoid hyperkeratosis of the nipple and the areola (NHNA) is a rare, idiopathic, and benign dermatological condition of the nipple and areola.

Epidemiology

Most often seen in females of reproductive age, especially during the 2^nd and 3^rd decades of life. Less than 70 cases have been reported till now.

Clinical presentation

Patients most often are asymptomatic but the lesions may be itchy. Breastfeeding may pose a problem in the affected females. The lesions are characterized by hyperkeratotic, verrucous, hyperpigmented papules and plaques and “café au lait” pigmentation of the nipple and areola. It may involve the nipple, the areola, or both.

Pathology

Etiology is unknown. A change in the estrogen milieu has been proposed as a possible mechanism. Histopathologically, it shows hyperkeratosis, filiform acanthosis, papillomatosis, and keratin plugging.

Treatment and prognosis

The main cause for concern in patients of NHNA is the cosmetic appearance of the nipple and areola. There is no definitive treatment; however, the various treatments that have been used are topical retinoic acid, topical calcipotriol, low-dose acitretin, cryotherapy, carbon dioxide laser, shave excision, and removal of the areola and reconstruction with a skin graft.

Breast function is threatened and it leads to bad aesthetic results, which often lead to mismanagement.

History and etymology

It was first described in the medical literature by Tauber in 1923.

Differential diagnosis

Possible considerations include:

• epidermal nevus

• acanthosis nigricans

• chronic eczema

• atopic eczema

• seborrheic keratosis

• Darier disease

• Paget disease

• basal cell carcinoma

• dermatophytosis

• Bowen disease