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Hyperkeratosis of nipple and areola
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Hyperkeratosis of nipple and areola

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Contributors: Ailia Ali MD, Susan Burgin MD
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Synopsis

Hyperkeratosis of the nipple and areola (HNA), formerly known as nevoid hyperkeratosis of the nipple and areola, is a rare benign skin condition that presents with verrucous thickening and hyperpigmentation of the nipple and/or areola. HNA can be unilateral or bilateral. Some patients may experience associated pruritus. The skin lesions are painless and without discharge; they are not associated with any lymphadenopathy.

HNA may be primary or occur secondary to the presence of a seborrheic keratosis or epidermal nevus, or as part of an underlying dermatosis such as Darier disease, acanthosis nigricans, atopic dermatitis, inherited ichthyoses (eg, ichthyosis vulgaris), or confluent and reticulated papillomatosis of Gougerot and Carteaud (CARP). Some cases have been ascribed to medications (estrogens, spironolactone, and vemurafenib).

HNA is seen in both sexes, but the majority of cases have been reported in postpubescent females, mainly those in their second to third decade of life. To date, there have been approximately 150 cases reported in the literature, but it is highly likely that this condition is underreported. A single case has been reported in a prepubescent girl.

Pathophysiology of primary HNA is unclear, although the occurrence of HNA mostly in female patients after puberty, its worsening in pregnancy with improvement post-delivery, and its occurrence in patients on estrogen therapy suggest an underlying hormonal pathogenesis.

Codes

ICD10CM:
N64.59 – Other signs and symptoms in breast

SNOMEDCT:
403459000 – Hyperkeratosis of nipple

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 05/30/2018
Last Updated: 05/06/2019
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Hyperkeratosis of nipple and areola
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Hyperkeratosis of nipple and areola : Pruritus, Hyperpigmented plaques
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