Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferences

View all Images (6)

Hyperkeratosis of nipple and areola
Other Resources UpToDate PubMed

Hyperkeratosis of nipple and areola

Contributors: Ailia Ali MD, Susan Burgin MD
Other Resources UpToDate PubMed

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

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

To perform a comparison, select diagnoses from the classic differential

Subscription Required

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

Drug Reaction Data

Subscription Required

References

Subscription Required

Last Reviewed:05/29/2018
Last Updated:05/05/2019
Copyright © 2024 VisualDx®. All rights reserved.
Hyperkeratosis of nipple and areola
A medical illustration showing key findings of Hyperkeratosis of nipple and areola : Pruritus, Currently pregnant, Hyperpigmented plaques
Copyright © 2024 VisualDx®. All rights reserved.