Abstract
INTRODUCTION: Nevoid hyperkeratosis of the nipple and/or areola (NHNA) is a benign lesion with a female predominance and an aesthetically disturbing appearance. Spontaneous remission is not reported and medical treatments proposed so far have shown variable results. PRESENTATION OF CASE: We describe a rare case of an extensive variant of NHNA covering almost the entire breasts' surface. At present, only three other reports are present in the literature. Medical treatment proved not completely effective and the patient was also affected by a significant breast asymmetry-hypertrophy. Therefore, NHNA was managed surgically with excision of the areolar affected portions while performing breast reduction-lift. The result was satisfactory and without recurrence of lesions at 5-year follow-up. DISCUSSION: This case reported favorable outcomes of surgery for NHNA. Reports of success with these procedures are still limited, but the promising results in terms of radicality and aesthetic outcome suggest it should be offered to patients as a viable therapeutic option. CONCLUSION: Indications for surgical treatment of NHNA can be: unsatisfying response to topical agents; young patients who want to restore the aesthetic appearance of the breast; and patients with concomitant indication for corrective surgery of the breast. Advantages are: predictable time of healing; predictable final result; radical excision of the affected tissue; and possibility of histologic analysis of the whole areola. In rare cases of lesions extending to the breast, preliminary treatment with topical agents can limit the extent of excision. Management and treatment should always be tailor-made for each individual case.