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.
Treatment and prognosis
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.
History and etymology
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
- Bowen's disease
- 1. Foustanos A, Panagiotopoulos K, Ahmad D et-al. Surgical approach for nevoid hyperkeratosis of the areola. J Cutan Aesthet Surg. 2012;5 (1): 40-2. doi:10.4103/0974-2077.94343 - Free text at pubmed - Pubmed citation
- 2. Verma P, Pandhi D, Yadav P. Unilateral Nevoid/primary Hyperkeratosis of Nipple and Areola Successfully Treated with Radiofrequency Ablation. J Cutan Aesthet Surg. 2011;4 (3): 214-5. doi:10.4103/0974-2077.91258 - Free text at pubmed - Pubmed citation
- 3. Ghanadan A, Balighi K, Khezri S et-al. Nevoid Hyperkeratosis of the Nipple and/or Areola: Treatment with Topical Steroid. Indian J Dermatol. 2013;58 (5): 408. doi:10.4103/0019-5154.117347 - Free text at pubmed - Pubmed citation
- 4. Nicholson BT, Harvey JA, Cohen MA. Nipple-areolar complex: normal anatomy and benign and malignant processes. Radiographics. 2009;29 (2): 509-23. doi:10.1148/rg.292085128 - Pubmed citation