What is Hyperkeratosis of the Nipple and Areola?
Hyperkeratosis of the nipple and areola is an uncommon and non-harmful skin condition. It is characterized by dark and thickened skin, which can look warty, on the nipple, the areola (the ring of pigmented skin around the nipple), or both. There are known variations of this condition, including nevoid hyperkeratosis of the nipple and areola, and pregnancy-associated hyperkeratosis of the nipple. When we talk about ‘nevoid’, we’re referring to coming from or resembling a mole or birthmark, and ‘pregnancy-associated’ means the condition is related to pregnancy.
What Causes Hyperkeratosis of the Nipple and Areola?
Hyperkeratosis of the nipple and areola, which means the thickening of the skin in these areas, happens for reasons we don’t fully understand yet. However, certain medications have been linked to this condition in some reported cases. These include estrogens (commonly used for prostate cancer), spironolactone, and some anticancer medicines, such as vemurafenib. It’s important to note, no viral DNA has been found in the skin lesions that accompany this type of hyperkeratosis, suggesting that it is not caused by a virus.
Risk Factors and Frequency for Hyperkeratosis of the Nipple and Areola
Hyperkeratosis of the nipple and areola is a skin condition that can appear in both males and females. However, it’s observed more often in females, accounting for about 80% of cases. Most of these cases occur in their twenties or thirties. Until now, around 150 cases have been reported. The true number might be higher though, because this condition doesn’t always cause symptoms. As a result, many people may not feel the need to see a doctor.
Signs and Symptoms of Hyperkeratosis of the Nipple and Areola
Hyperkeratosis of the nipple and areola is a skin condition that can appear on one or both nipples and areolas. In some rare situations, it can even spread beyond these areas. The skin changes are generally not painful and don’t secrete anything or cause affected lymph nodes to swell. Some patients might experience itchiness. In men who are taking estrogen therapy, breast enlargement may occur at the same time with this skin condition.
This condition usually develops during puberty or pregnancy. It’s characterized by more pigmentation and a rough, wart-like thickening of the affected areas. These skin changes might get darker and thicker during pregnancy, but often improve after childbirth. The degree of skin thickening can vary from patient to patient. In some situations, breastfeeding might become difficult if the nipples are affected significantly. This condition is not known to start before puberty.
There are two primary types of classifications proposed for this condition. The first was suggested by Levy-Franckel and classifies it into three types:
- An epidermal nevus type, involving one side of a single breast, usually appearing in a linear pattern, seen equally in both sexes.
- A type associated with a skin condition called ichthyosis, involving both breasts, also seen equally in both sexes.
- A type affecting both the nipples and areolas of both breasts, primarily seen in women in their twenties or thirties.
The second classification system proposes two types:
- A primary or idiopathic type.
- A secondary type associated with other skin conditions such as a nevus (commonly epidermal nevus or organoid nevus leiomyomas), a warty tumor, ichthyosis, chronic eczema, Darier disease, acanthosis nigricans, terra firma dermatitis, a certain type of dermatitis, and a form of skin lymphoma.
The second type could also include breast changes in pregnant women and men receiving estrogen therapy. During pregnancy, normal changes like pigmentation and enlarging of the nipple and areola should be considered when diagnosing this condition.
Distinguishing between the primary (idiopathic) and secondary types of hyperkeratosis of the nipple and areola can be a challenge. This is because they often have similar histological appearances with other skin conditions such as seborrheic keratosis, acanthosis nigricans, and epidermal nevus. These conditions may present similar histological features but vary in other aspects such as the areas of the body they typically affect.
Testing for Hyperkeratosis of the Nipple and Areola
In cases of a condition called hyperkeratosis of the nipple and areola (thickening and hardening skin on the nipple and the surrounding circle), examinations like mammograms and breast ultrasounds can usually show no irregularities or issues.
Treatment Options for Hyperkeratosis of the Nipple and Areola
Hyperkeratosis of the nipple and areola is a condition that doesn’t go away without treatment and often lasts a long time. Different kinds of medical treatments or surgeries can be used to help with this.
Keratolytic topicals, which are lotions or creams that help to remove the thick outermost layer of the skin, are usually the first option for treatment. These contain ingredients such as salicylic acid or lactic acid. However, these are rarely enough to completely resolve the condition.
Creams called topical corticosteroids can help relieve itching but don’t do much else. Another type of cream, called topical retinoids, which contain substances like tretinoin 0.05% and isotretinoin, has been used with limited success. There’s also a cream called calcipotriol, a type of vitamin D that regulates skin growth and change, but it’s not very effective.
A cream that combines hydroquinone with kojic acid may help lighten the color of the breast skin, but it won’t improve the thickened, rough texture characteristic of hyperkeratosis. Certain systemic (whole body) retinoid treatments were also found ineffective.
However, more physical types of treatments can give better results. These include cryotherapy with liquid nitrogen, which freezes off the affected skin, and carbon dioxide laser treatment. Both of these destroy the thickened skin. Another option is surgical removal of the affected areas. Surgical choices can include shave excision (removing the thickened skin with a razor-like tool), curettage (scraping away the affected skin), and areola removal with skin graft reconstruction (replacing the removed areola with a graft of skin from another part of the body). In two cases, a method called tangential excision, using a radiofrequency surgical device to cut off the affected skin, was used. These two patients did not have their hyperkeratosis come back for 7 to 9 months after the treatment.
What else can Hyperkeratosis of the Nipple and Areola be?
If you have hyperkeratosis of the nipple and areola (a condition causing thickened skin on the nipple and surrounding area), doctors have to consider several possible diagnoses, including:
- Fox Fordyce disease
- Hyperkeratosis resulting from a long-lasting friction
- Mycosis fungoides affecting the nipple and areola
- Pigmented basal cell carcinoma
- Seborrheic keratosis (a type of skin growth)
