Nipples develop on the milk lines of mammals. Most humans have two nipples*, but sometimes more than two develop along these lines.
Hyperkeratosis of the nipple and/or areola is
not a single disease entity and presents as localised or diffuse verrucous thickening
and brownish discolouration of the nipple and/or areola. It can be aetiologically classified into secondary or primary (idiopathic) types.
Different
conditions come under the umbrella term “secondary hyperkeratosis of the nipple
and/or areola”. This secondary type can be unilateral or bilateral depending on the diagnosis
and the conditions reported were various such as pregnancy, chronic eczema,
seborrhoeic keratosis, acanthosis nigricans, Darier’s disease, verrucous
epidermal naevus, ichthyosis, cutaneous T-cell lymphoma, HPV infection, Malassezia infection**, oestrogen therapy and sorafenib therapy.
The histopathological findings obviously depend on the diagnosis. In the first two patients (first five photos), the hyperkeratosis was due
to warts. In the third patient (sixth and seventh photos), it was due to acanthosis nigricans associated with obesity.
Pseudohyperkeratosis of the nipple and areola can result from inadequate
hygiene (dermatitis neglecta).
Primary hyperkeratosis of the nipple
and/or areola*** is usually bilateral and occurs predominantly in females in the
second or third decade of life.
Histopathologically it shows orthokeratotic hyperkeratosis, papillomatosis, interconnecting acanthosis and keratin plugging. An inverse form of primary hyperkeratosis of the nipple and/or areola was described where the lesions spared the nipples and sparsely affected the areolae, with the majority of the lesions extending to the adjacent periareolar skin, covering the entire breast. Paget’s
disease of the nipple and areola must be
excluded and in doubtful cases, biopsy is required. Imaging studies should be performed whenever there is any concern about an
underlying breast disease. Another
condition that is commonly misdiagnosed as Paget's disease of the nipple and
areola or eczema of the nipple and areola is erosive adenomatosis of the
nipple. Fox-Fordyce disease should also be considered in the
differential diagnosis. It may produce itchy papules on the areola.
Several therapeutic modalities have been
suggested for hyperkeratosis of the nipple and areola such as keratolytic
therapy, topical calcipotriol, topical isotretinoin, excision with or without
grafting, laser therapy, and cryotherapy. The choice depends on the whether it
is idiopathic or secondary and the cause of the secondary type. In the first patient (first four photos), cryotherapy was chosen in
view of the diagnosis and has proved to be effective.
 |
Two warts
on the left nipple (perianal warts were also found)
|
 |
Healing taking place, six days after cryotherapy of the lesions |
 |
Almost complete healing after cryotherapy of the lesions |
Complete healing 3 months after cryotherapy of the lesions
Sexually transmitted nipple warts
Acanthosis nigricans
Acanthosis nigricans of the right areola
*Milk lines:
Nipples develop on the milk lines of mammals. Most humans have two nipples, but sometimes more than two develop along these lines.
**Li C, Ran Y, Sugita T, Zhang E, Xie Z, Cao L. Malassezia associated hyperkeratosis of the nipple in young females: Report of three cases. Indian J Dermatol Venereol Leprol 2014;80:78-80.
***Verma P, Pandhi D, Yadav P. Unilateral Nevoid/primary hyperkeratosis of nipple and areola successfully treated with radiofrequency ablation. J Cutan Aesthet Surg. 2011; 4:214-5.
This page was last updated in November 2017.