Hyperkeratosis of the Nipple and Areola
- Author: Joseph C English III, MD; Chief Editor: Dirk M Elston, MD more...
Background
Hyperkeratosis of the nipple and/or areola (HNA) is defined as excessive keratinization of the nipple and/or areola. Hyperkeratosis of the nipple and/or areola is characterized by hyperpigmented, verrucous or filiform, keratotic thickening of the nipple and/or areola, with a papillomatosis or velvety sensation to touch. Hyperkeratosis of the nipple and/or areola has classically been classified into the following 3 subsets[1] :
- Type I - hyperkeratosis of the nipple and/or areola due to the extension of an epidermal nevus
- Type II - hyperkeratosis of the nipple and/or areola in conjunction with disseminated dermatoses
- Type III - Nevoid hyperkeratosis of the nipple and/or areola
Alternative classifications schemes have been suggested.[2, 3, 4] Upon review of the literature, a recommended classification is into (1) primary hyperkeratosis of the nipple and/or areola, which is idiopathic,[5, 6, 7, 8, 9] and (2) secondary hyperkeratosis of the nipple and/or areola, which is associated with the following:
- Epidermal nevus
- Organoid nevus[10]
- Leiomyomas[11]
- Verruca[12]
- Malignant acanthosis nigricans[13]
- Chronic eczema such as atopic dermatitis
- Pregnant females
- Males receiving hormonal therapy for prostate cancer[18, 19]
- Estrogen therapy for androgen insensitivity syndrome[20]
Pathophysiology
The pathophysiology of hyperkeratosis of the nipple and/or areola is unknown.
Epidemiology
Frequency
United States
Hyperkeratosis of the nipple and/or areola is rare, and currently no domestic incidence rates have been documented in the literature.
International
Hyperkeratosis of the nipple and/or areola is rare, and currently no international incidence rates have been documented in the literature.
Mortality/Morbidity
Mortality is not associated with primary hyperkeratosis of the nipple and/or areola. The morbidity rate is low, and morbidity is primarily limited to the undesirable cosmetic results of the abnormal nipple and/or areola. The morbidity and mortality rates of secondary hyperkeratosis of the nipple and/or areola are those of the underlying diseases; thus, the rates with secondary hyperkeratosis of the nipple and/or areola may be greater than those with other types of hyperkeratosis of the nipple and/or areola.
Race
Hyperkeratosis of the nipple and/or areola has no reported racial predilection.
Sex
Hyperkeratosis of the nipple and/or areola is more common in females than in males. In a study of 45 primary hyperkeratosis of the nipple and/or areola patients 80% were females.[21]
Age
In females, hyperkeratosis of the nipple and/or areola most commonly occurs in those aged 10-40 years. Males with nevoid hyperkeratosis of the nipple and/or areola are often older than females, but no specific age distribution is reported.
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