A Rare Case of Nevoid Hyperkeratosis of the Nipple and Areola: A Diagnostic Dilemma

Abstract Nevoid hyperkeratosis of the nipple and areola (NHNA) is an uncommon, benign dermatological condition characterized by verrucous hyperpigmented plaques involving the nipple-areola. We report a case of a 30-year-old female patient who presented with a 2-year history of hyperpigmented, raised lesion over the left nipple and areola. On clinical examination, a well-defined, hyperkeratotic, verrucous plaque localized to the left areola was noted. Dermoscopy showed thickening with multiple gray–black globules. Histopathology demonstrated hyperkeratosis, acanthosis, elongated filiform rete ridges, and chronic lymphocytic infiltrate. Based on clinical, dermoscopic, and histological features, a diagnosis of NHNA was established. Recognition of NHNA is essential to distinguish it from mimickers such as Paget’s disease, basal cell carcinoma, and other neoplastic or infectious conditions affecting the nipple and areola. Early diagnosis prevents unnecessary interventions and addresses cosmetic concerns effectively.