History
A 68-year-old patient presents with a 3-year history of a rash surrounding her right nip-ple, which is red and mildly pruritic. She denies any discharge from the nipple. Topical steroids prescribed by her GP had been ineffective so she was referred to the dermatol-ogy out-patient clinic. The patient has a history of eczema as a child and asthma. More recently she has developed irritation on her hands since looking after her grand daughter 3 days per week. She is otherwise well and takes hormone replacement therapy.
Examination
There is a sharply demarcated erythematous plaque surrounding the right nipple with some slight overlying scale, measuring 6 cm in diameter (Fig. 62.1). There is no obvious underlying breast mass or axillary lymphadenopathy. The left nipple is normal and full skin examination does not reveal any further similar areas. On the dorsi of her hands she has an eczematous rash in the finger webs and under her wedding ring.
Questions
• What is the most likely diagnosis?
• How would you investigate this patient?
• What treatment options are possible?
Medicine
Asymmetrical ‘eczema’ of the nipple that is unresponsive to topical steroids should always raise the possibility of an underlying malignancy of the breast. Patients should be referred for consideration of an urgent skin biopsy. This patient was diagnosed with Paget’s disease of the right nipple. This represents the intra-epidermal spread of an intraductal breast carcinoma. Although clinically this may mimic eczema a high index of suspicion is needed if the patient is elderly, the disease is asymmetrical and largely unresponsive to conventional eczema therapy. This patient did have a history of atopy; however, atopic eczema of the nipple, although very common, is usually symmetrical and responds rapidly to topical corticosteroids.
Paget’s disease is uncommon and involves the nipple or areola, and manifests as ery-thematous, well-demarcated plaques with overlying scale. It is most commonly seen in woman over 50 years of age. Onset, as in this case, is usually very insidious. Lesions can be asymptomatic or pruritic, painful, oedematous, bleeding or ulcerated. Sometimes nipple retraction and discharge may occur. An underlying breast mass is palpable in less than 50 per cent of patients. A skin biopsy should be performed from the affected skin. A mammogram and further work-up of any palpable breast mass is vital.
As with any other breast cancer, treatment consists of surgery, radiotherapy and/or chemotherapy. Prognosis is variable and is worse when associated with an underlying breast mass and lymph node involvement. A similar condition called extramammary Paget’s disease may occur away from the nipple and present with a similar eczema-like eruption, usually around the female/male anogeni-tal region or axilla. It is histologically similar to Paget’s disease and may be associated with intra-epidermal spread of a ductal apocrine carcinoma of the lower gastrointestinal tract, urinary or female genital tract.
KEY POINTS
• Paget’s disease should be suspected when presented with asymmetrical ‘eczema’ of the nipple.
• An underlying breast mass is palpable in less than 50 per cent of patients.
• Extramammary disease occurs mainly around the female/male anogenital region.
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