Paget’s Disease of the Breast: Symptoms, Causes
Paget’s disease of the breast is a rare condition associated with underlying breast cancer. Learn about its symptoms, causes
Paget’s Disease Of Breast
Paget’s disease is a rare form of breast cancer, accounting for less than 5% of breast cancer cases in women. Although uncommon, Paget’s disease can also affect men.
Paget’s disease of the breast is a rare form of breast cancer that affects the nipple and the surrounding areola. It was first identified by Sir James Paget in 1874 and accounts only 1–4.3% of all breast cancer cases.
The most common sign of Paget’s disease is a scaly, red rash on the nipple. In individuals with darker skin tones, redness may be more difficult to recognize. The rash may be accompanied by itching or a burning sensation. The darker area of skin surrounding the nipple (the areola) may also become involved.
This condition is also referred to as Paget’s disease of the nipple. It originates in the skin of the nipple and areola and is characterized by the presence of distinctive malignant cells known as Paget cells.
Paget’s disease can resemble eczema or other benign skin conditions; however, there are important differences. While eczema of the breast typically affects the areola first and then spreads to the nipple, Paget’s disease usually begins at the nipple and may subsequently extend to the areola.
In the majority of patients (92–100%), mammary Paget’s disease is associated with an underlying breast cancer, either ductal carcinoma in situ (DCIS) or invasive breast cancer. About half of these patients also have a palpable lump in the breast.
Related Article: Rare Type of Breast Cancer
Paget’s disease is Ductal carcinoma in situ and/or Invasive ductal cancer?
The prognosis of Paget’s disease of the breast depends mainly on presence or absence of invasive breast cancer is present.
Paget’s disease is either ductal carcinoma in situ (DCIS) and/or invasive ductal carcinoma.
When a palpable breast mass is present, it is more likely that the patient is suffering from invasive carcinoma.
In contrast, patients who do not have a clinically detectable mass are more likely to have ductal carcinoma in situ (DCIS).
The associated tumors may be palpable or non-palpable, are usually located near the areola, tend to be centrally positioned, and are often multifocal. However, tumors located in the peripheral areas of the breast have also been reported.
Paget’s disease Symptoms
Initial stage
1. The most common presenting feature of Paget’s disease is a red (erythematous) lesion of the nipple, which may extend to involve the surrounding areola.
2. The affected breast skin may appear pigmented, thickened, and may show weeping or crusted lesions with irregular borders.
3. Bleeding from the nipple or breast, breast pain, and itching, burning and tingling are also common features.
4. Early symptoms, such as scaling and redness, may resemble eczema or other inflammatory skin conditions. When treated with topical steroid medications, these symptoms may temporarily improve, which can mask the underlying disease and delay proper diagnosis and treatment.
Advance stage
1. As Paget’s disease progresses, it may lead to ulceration and destruction of the nipple–areola complex, and nipple retraction may occur. This clinical presentation is distinct from inflammatory breast carcinoma, which typically shows diffuse redness of the breast with secondary involvement of the skin and nipple.
2. In advanced stages, patients may experience ulceration, bleeding from the nipple, serous discharge, and extensive destruction of the nipple–areola complex.
3. The lesion is usually confined to the nipple or may extend to the areola; in advanced cases, it can also involve the surrounding breast skin.
4. The surface of the lesion may appear slightly thickened or infiltrated.
5. Breast pain or itching become more frequent.
6. Advanced lesions are characterized by pink or red, circular, oval, or polycyclic eczema-like plaques that are clearly separated from the surrounding normal skin.
7. Paget’s disease lesions are usually unilateral and rarely bilateral. The disease may also develop in ectopic breast tissue or accessory nipples.
8. Nipple retraction or inversion is often described as a symptom of Paget’s disease; however, flattening of the nipple is more common, and true nipple retraction usually occurs due to an underlying breast tumor.
Paget’s disease Risk factor
Gender: Paget’s disease is much more common in women. However, men can also develop Paget’s disease of the breast.
Age: It occurs most often in postmenopausal women (average age around 57 years). Nevertheless, cases have been reported in adolescents as well as elderly individuals.
Race: Because Paget’s disease causes skin changes, its appearance may vary across different ethnic groups. In individuals with darker skin, redness (erythema) may appear violaceous or less noticeable, which can sometimes lead to delayed recognition.
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