This ulcerating breast lesion is Paget's disease of the nipple, a chronic eczematous lesion of the
nipple-areola complex, first described by Velpeau in 1856. He, however, failed to recognize the
association between the nipple lesion and an underlying breast cancer. In 1874, Sir James Paget described
fifteen patients with identical nipple changes all of whom subsequently developed breast cancer.
Paget's disease usually presents as a well demarcated, erythematous, exudative lesion, starting on the nipple
and spreading onto the adjacent areola. Progression of the disease leads to ulceration and destruction of the
nipple. The commonest symptoms are a bloody nipple discharge (40%), pain (30%) and itch (15%). In 10% of
patients the lesion is asymptomatic. Paget's disease is almost invariably associated with a ductal carcinoma.
About 50% patients have an associated palpable breast lump and an invasive tumour. In the 50% without a palpable
lesion there is usually high-grade ductal carcinoma in-situ (DCIS). Any eczematous or vesicular
nipple lesion should be regarded with suspicion. The differential diagnosis includes chronic eczema, intraduct
papilloma, malignant melanoma, Bowen's disease and duct ectasia. All patients should undergo mammography and the
diagnosis can be confirmed or excluded by a full-thickness nipple biopsy.
Histologically the diagnosis is confirmed by the finding of Paget cells - large round cells with pale
cytoplasm and pleomorphic nuclei - within the epidermis. Until recently the histiogenesis of these cells was
uncertain. Some believed that they were ductal carcinoma cells migrating along the basement membrane. Others
considered them to be malignant keratinocytes or melanocytes transformed in-situ and possibly
independent from the underlying cancer. Histochemical studies have shown
the cells to be derived from glandular epithelium and to be immunologically similar to the underlying cancer
Until recently the surgical management of Paget's disease invariably consisted of mastectomy and axillary
clearance irrespective of the presence or absence of a palpable lump. It is now accepted that the management of
Paget's disease with a palpable lump should be that of the underlying invasive cancer. With the increased
understanding of the behaviour of DCIS, there has been a move towards more conservative treatment of Paget's
disease without a palpable lesion. If these patients have a normal mammogram excision of the nipple-areola
complex combined with post-operative radiotherapy has been reported on a small numbers of patients with low
local recurrence rates. If a mammogram shows microcalcification a conservative approach can be adopted but it
should be noted that DCIS is often extensive and multifocal and that histologically clear margins should be
achieved. If the surgical resection margins are involved then mastectomy should be performed. If recurrence
occurs following conservative treatment for DCIS then in over 50% cases this recurrence is invasive. The exact
management of Paget's disease in association with DCIS will depend very much on the risks that the surgeon and
patient is prepared to take in order to preserve the breast.
Not surprisingly the prognosis of Paget's disease depends on the underlying breast disease. For those with a
palpable lesions, 5 and 10-year survival rates of 40% and 20% respectively have been reported. For those without
a palpable lump the prognosis is much better with 80% 10-year survival reported. There is no difference in
survival of patients with invasive breast cancer with or without Paget's disease.
Bijker N, Rutgers E J T, Duchateau L et al. Breast-conserving therapy for Paget's
disease of the nipple. Cancer 2001; 91: 472-477.
Jamali F R, Ricci A, Deckers P J. Paget's disease of the nipple-areola complex. Surg
Clin North Am 1996: 76: 365-381.