Saturday, 13 October 2012

Secondary hyperkeratosis of the nipple

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 PPandhi DYadav 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

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Main Works of Reference List (The first eight are my top favourites)

  • British National Formulary
  • British National Formulary for Children
  • Guidelines (BAD - BASHH - BHIVA - Uroweb)
  • Oxford Handbook of Genitourinary Medicine, HIV, and Sexual Health
  • Oxford Handbook of Medical Dermatology
  • Rook's Textbook of Dermatology
  • Simple Skin Surgery
  • Weedon's Skin Pathology
  • A Concise Atlas of Dermatopathology (P Mckee)
  • Andrews' Diseases of the Skin
  • Andrology (Nieschlag E FRCP, Behre M and Nieschlag S)
  • Bailey and Love's Short Practice of Surgery
  • Davidson's Essentials of Medicine
  • Davidson's Principles and Practice of Medicine
  • Fitzpatrick's Colour Atlas and Synopsis of Clinical Dermatology (Klaus Wolff FRCP and Richard Allen Johnson)
  • Fitzpatrick’s Dermatology in General Medicine
  • Ganong's Review of Medical Physiology
  • Gray's Anatomy
  • Hamilton Bailey's Demonstrations of Physical Signs in Clinical Surgery
  • Hutchison's Clinical Methods
  • Lever's Histopathology of the Skin
  • Lever's Histopathology of the Skin (Atlas and Synopsis)
  • Macleod's Clinical Examination
  • Martindale: The Complete Drug Reference
  • Oxford Handbook of Clinical Examination and Practical Skills
  • Oxford Textbook of Medicine
  • Practical Dermatopathology (R Rapini)
  • Sexually Transmitted Diseases (Holmes K et al)
  • Statistics in Clinical Practice (D Coggon FRCP)
  • Stockley's Drug Interactions
  • Treatment of Skin Disease: Comprehensive Therapeutic Strategies
  • Yen & Jaffe's Reproductive Endocrinology