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.

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

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.

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

The main cause for concern in patients of NHNA is the cosmetic appearance of 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.

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

Possible considerations include:

  • epidermal naevus
  • acanthosis nigricans
  • chronic eczema
  • atopic eczema
  • seborrheic keratosis
  • Darier's disease
  • Paget's disease
  • basal cell carcinoma
  • dermatophytosis
  • Bowen's disease
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Article Information

rID: 27147
System: Breast
Section: Pathology
Tag: breast
Synonyms or Alternate Spellings:
  • Nevoid hyperkeratosis of the nipple and the areola (NHNA)
  • NHNA

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