Hyperkeratosis of the Nipple and Areola Workup

Updated: May 02, 2017
  • Author: Rabindranath Nambi, MD; Chief Editor: Dirk M Elston, MD  more...
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Workup

Laboratory Studies

No blood or urine laboratory tests aid in the diagnosis of primary hyperkeratosis of the nipple and/or areola (HNA).

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Imaging Studies

Imaging studies must be performed if any concern about underlying breast disease exists. Mammography should be performed, with or without ultrasonography. Ultrasonography of the nipple may demonstrate skin thickening. [29]

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Other Tests

In cases of secondary hyperkeratosis of the nipple and/or areola, additional testing may be required. Malignant acanthosis nigricans associated hyperkeratosis of the nipple and/or areola requires possible endoscopy and/or colonoscopy and CT scanning. Cutaneous T-cell lymphoma–associated blood testing for clonality and CT scanning for staging may need to be performed.

Dermoscopy findings have mimicked a pigmented basal cell carcinoma in one case, with multiple blue-gray globules and leaflike areas. [30]

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Procedures

The acquisition of a skin biopsy sample for histopathologic examination aids in differentiating nevoid hyperkeratosis of the nipple and/or areola from other conditions. The preferred method involves a 3- or 4-mm punch biopsy followed by closure with 6-0 Prolene sutures. An alternative suture material (eg, 5-0 or 6-0 plain gut [absorbable] suture) may be used if desired. This method provides the best cosmetic results, with minimal scarring and maintenance of the normal architecture of the nipple and/or areola.

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Histologic Findings

Primary hyperkeratosis of the nipple and/or areola is characterized by variable orthokeratotic hyperkeratosis, slight acanthosis, and marked papillomatosis changes on routine hematoxylin and eosin–stained specimens. Additional findings reported include mild dermal lymphocytic perivascular inflammation [27, 31, 32] and epidermal spongiosis with microabscesses with normal lymphocytes. [33, 34] Histopathological features and an immunophenotype that paralleled those of mycosis fungoides have been reported. [34, 35] Immunostaining demonstrated epidermal infiltrate with CD3, a predominance of CD4 compared with CD8, and absence of CD7 expression. However, in both cases, no clonal T-cell population was identified.

In secondary hyperkeratosis of the nipple and/or areola, biopsy samples may reveal histologic findings related to the associated skin disease. An example includes cutaneous T-cell lymphoma–associated hyperkeratosis of the nipple and/or areola histology, which reveals epidermotropism with atypical lymphocytes. [17]

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