Erosive adenomatosis of the nipple masquerading as Paget's disease

Abstract

Erosive adenomatosis of the nipple (EAN) is a rare, benign neoplasm of breast lactiferous ducts. Peak incidence is in the fifth decade in women. Clinically, it is mistaken for eczema during early stages and Paget's disease at later stages. We present a young lady with EAN who was advised mastectomy. It is important for dermatologists to be familiar with this entity to avoid topical steroid abuse and unwarranted breast removal. This case is presented for awareness and importance of correct and early diagnosis.

INTRODUCTION

Erosive adenomatosis of the nipple (EAN) was first defined by Jones in 1955 who referred to it as florid papillomatosis or papillary adenoma of the nipple. In 1959 LeGal, et al. coined the term erosive adenomatosis because of the clinical and pathologic findings. Adenoma of the nipple, sub areolar papillomatosis, papillary adenoma of the nipple, and florid sub areolar duct papillomatosis are some of the other terms to describe EAN. EAN, a benign neoplasm, is common after the fifth decade but has been reported in children.[1] EAN is often suspected as Paget's disease clinically and misinterpreted as syringadenoma papilliferum, hidradenoma papilliferum, or low-grade adenocarcinoma histologically, but it has received almost no notice by the dermatologists. Recognition of this entity is important because of clinical and therapeutic implications.

CASE REPORT

A 23-year-old unmarried woman presented with history of oozing of serous and very rarely sanguineous discharge from right nipple for more than a year. There was no definite history of exacerbation in relation with menstrual cycles, which were normal. There was no family history of similar illness or carcinoma of breast. On examination, the skin over the nipple was rough and thickened with erosion and superficial fissuring [Figure 1]. The examination of the breasts and regional nodes were normal. The ultrasonogram done after a course of antibiotics was normal. She was diagnosed with EAN and treated with antibiotics and short-term mild topical steroids with remission but was not sustained. When she saw discharge of frank blood, the alarmed patient sought opinion from various specialists and was advised mastectomy by some of them. A biopsy of the nipple was performed, which showed features of EAN [Figures 2 and 3]. Being an unmarried girl, radiofrequency is being planned rather than excising the nipple. She will be followed up regularly for recurrence if any.

Figure 1.

Thick, rough skin of nipple with erosion and fissuring

Figure 2.

Low power-perpendicular tubular structures showing connection with the overlying eroded epidermis; dermis showing round, oval, few large or irregular shaped ducts lined by columnar epithelium [H & E, 40×]

Figure 3.

High power of a duct showing apocrine snouts [H & E, 100×]

DISCUSSION

EAN, a benign tumor of the nipple, is neoplasm of lactiferous duct. The pathogenesis is not very clear and there are two schools of thought regarding the origin of this proliferation of ducts. One concept being that the origin is considered to be a precursor of breast adenocarcinoma[2] and the other, a marker for fibrocystic breast changes.[3] A careful review and the available data indicate that the evidence for an association between EAN and breast carcinoma is not convincing. However, its relationship with fibrocystic breast changes is not proved beyond doubt.[1]

Histologically, EAN is characterized by a non-encapsulated proliferation of ducts. Two patterns have been observed: An adenomatous pattern consisting of a proliferation of round, oval or irregular-shaped ducts within a stroma, which may be normal, fibrotic or hyalinized. Ducts are lined by columnar epithelium and have an outer myoepithelial layer. Apices of the columnar cells show apocrine snouts. The second papillomatous pattern shows ducts that are larger, oval or round with increased cellularity. Intraluminal growth of the inner cell layer may almost occlude the lumen.[4] These 2 patterns seem to represent a spectrum, with most cases having some degree of overlap. Differentiating the histological pattern from carcinoma is very important. In our case, the absence of cytological atypia, the 2 cell types lining the ducts, and intraductal papillomatosis differentiates it from carcinoma. The organized pattern of the ducts and absence of prominent inflammatory reaction rules out syringocystadenoma papilliferum. The connection of tubular structure with the overlying eroded epidermis rules out possibility of hidradenoma papilliferum. Partial or complete nipple excision is the recommended treatment of choice, which is not without chance of recurrence. Treatment consists of local excision of the nipple and plastic reconstruction. Unnecessary mastectomy or extensive surgery must be avoided. A recent report has described a successful treatment with cryosurgery. It was postulated that cryosurgery, as used in the treatment of other benign conditions, could eradicate the tumor through local destruction. Deeper necrosis, as required for treatment of a malignancy, would not be needed. Although a single treatment may suffice, re-treatment can be safely carried out.[5] The usefulness of radiofrequency in EAN has not been reported. Being an unmarried woman, absence of other clinical findings in the breasts and with a normal ultrasonogram, the authors hope and wish that the second school of thought, that EAN be a reaction pattern to fibrocystic breast changes, as suggested by Perzin, et al., is the cause for the origin in this patient. Cryosurgery is planned for the patient and she will be followed up regularly. The patient and the family are counseled enough and adequately re-assured of the benign nature of the disease.