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Case Reports

Abstract

Hyperkeratosis of the nipple and areola (HNA), aka. nevoid hyperkeratosis of the nipple and areola and pregnancy-associated hyperkeratosis of nipple is a rare benign condition, is characterized by unilateral or bilateral, usually painless verrucous hyperpigmentation and thickening of the nipple-areolar complex. It may be idiopathic or secondarily associated with hormonal changes. Majority of cases have been reported in young women and is often chronologically related to puberty, pregnancy, lactation, or estrogen therapy. In rare instances, drugs such as vemurafenib have also been implicated. HNA is frequently underrecognized and underdiagnosed due to its rarity and overlap clinically with dermatoses and histologically with acanthosis nigricans and sometimes, even mycosis fungoides. Here, we present three cases demonstrating the diverse clinicopathological presentations of this uncommon entity. Case#1: A 26-year-old pregnant woman in her second trimester presented with unilateral warty and eczematous nipple 0.5 x 1.0 cm lesion and bloody discharge. Case#2: A 36-year-old woman, not currently pregnant or lactating, presented with bilateral areolar skin thickening of 2-years duration. Case#3: A 33-year-old lactating woman presented with 5-month history of left nipple discoloration, bloody discharge and occasional pain. Biopsy on all cases revealed irregular epidermal hyperplasia, with filiform or blunted papillomatosis, hypergranulosis, hyperkeratosis and superficial dermal vascular ectasia and variable superficial perivascular lymphohistiocytic infiltrate. In case#2, intraepidermal microabscesses composed CD4+ lymphocytes with nuclear hyperchromasia and clonal TRBC1 expression (<25%) were present; thus, histopathologically overlapping with mycosis fungoides. Recognition of the variable clinicopathological features of this uncommon entity is essential for correct diagnosis and optimal patient management