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Hyperkeratosis of the nipple and areola

Author: Siska Falconer, Medical Student, Auckland University, Auckland, New Zealand. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. January 2020.


What is hyperkeratosis of the nipple?

Hyperkeratosis of the nipple and areola describes verrucous thickening of the nipples and areolae [1–5]. It can be primary (idiopathic) or secondary to another disorder (see differential diagnosis).

Hyperkeratosis of the nipple and areola is also called naevoid hyperkeratosis and hyperkeratosis areolae mammae naeviformis.

Hyperkeratosis of the nipple and areola

Who gets hyperkeratosis of the nipple?

Primary hyperkeratosis of the nipple typically presents in adolescent females, with the rates in males being much lower [1,2,4]. No ethnic or geographical links have been reported.

What causes hyperkeratosis of the nipple?

The exact cause of hyperkeratosis of the nipple is not known. Although no hormonal alterations have been found, an endocrinological cause has been proposed due to its association with the female sex, oestrogen therapy, and pregnancy [1].

What are the clinical features of hyperkeratosis of the nipple?

The clinical features associated with hyperkeratosis of the nipple follow.

  • The hyperkeratosis is located on the nipple and areola (although the entire breast can be affected) [1,5]
  • It is usually bilateral [1,2]
  • It presents as verrucous hyperpigmented plaques [1,4]
  • The plaques are generally asymptomatic; and occasionally itchy [1,3].

What are the complications of hyperkeratosis of the nipple?

Hyperkeratosis of the nipple may cause embarrassment and cosmetic concerns [5]. There are a few reported cases of difficulty breast-feeding from the affected breast [1].

How is hyperkeratosis of the nipple diagnosed?

Diagnosis of hyperkeratosis of the nipple relies upon the clinical presentation of the hyperkeratotic plaque(s) and findings on skin biopsy if the presentation is atypical.

Histopathological characteristics of hyperkeratosis of the nipple are:

  • Othokeratotic hyperkeratosis
  • Papillomatosis
  • Acanthosis
  • Keratotic plugging
  • A mild perivascular lymphocytic infiltrate in the dermis [1,4].

What is the differential diagnosis for hyperkeratosis of the nipple?

Secondary hyperkeratosis of the nipple [1,2,5] is usually unilateral. Examples include:

Unilateral hyperkeratosis of the nipple associated with pain, bleeding, ulceration, discharge, or nipple retraction should be investigated with breast examination, mammogram, and biopsy, in case of breast cancer.

Bilateral secondary hyperkeratosis of the nipple and areola may occur with:

What is the treatment for hyperkeratosis of the nipple?

Untreated hyperkeratosis of the nipple does not tend to interfere with the normal breast function. However, treatment of the lesion is generally warranted for cosmetic reasons [4,5].

Cryotherapy is a suitable first-line treatment for hyperkeratosis of the nipple often gives a cosmetically satisfactory result [4,5]. It may need to be repeated. Hypopigmentation is the main long-term risk of cryotherapy.

Medical treatments do not remove the lesions permanently but keratolytic agents (such as salicylic acid, lactic acid, or urea), topical retinoids, and calcipotriol, may be helpful [2,5].

Skin surgery or laser resurfacing is sometimes undertaken but may be cosmetically unsatisfactory [5].

What is the outcome for hyperkeratosis of the nipple?

Without treatment, hyperkeratosis of the nipple persists.

Cryotherapy or surgical excision reduces recurrence [4,5].

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References

  1. Shastry V, Betkerur J, Kushalappa PA. Unilateral naevoid hyperkeratosis of the nipple: A report of two cases. Indian J Dermatol Venereol Leprol 2006; 72: 303–5. PubMed
  2. Ayetekin S, Tarlan, N, Alp A, Uzunlar AK. Naevoid hyperkeratosis of the nipple and aerola. J Eur Acad Dermatol Venereol 2003; 17: 232–3. PubMed
  3. Samimi M, Maitre F, Esteve E. Hyperkeratotic lesion of the nipple revealing cutaneous leiomyoma. Ann Dermatol Venereol 2008; 135: 571–4. PubMed
  4. Kubota Y, Koga T, Nakayama J, Kiryu H. Naevoid hyperkeratosis of the nipple and areola in a man. Br J Dermatol 2001; 142: 382–4. PubMed
  5. Foustanos A, Panagiotopoulous K, Ahmad D, Konstantopoulous K. Surgical approach for naevoid hyperkeratosis of the aerola. J Cutan Aesthet Surg 2012; 5: 40–2. PubMed

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