2E65.5: Paget disease of nipple

ICD-11 code 2E65.5 refers to Paget disease of the nipple, a rare form of breast cancer that primarily affects the skin of the nipple and the areola. This condition typically presents as a scaly, red, and itchy rash on the nipple, which can sometimes be mistaken for eczema or dermatitis. Paget disease of the nipple often signals the presence of underlying breast cancer in the ducts of the breast.

While Paget disease of the nipple accounts for less than 5% of all breast cancer cases, it is important to diagnose and treat it promptly to prevent the spread of cancerous cells to other parts of the breast tissue or beyond. The most common treatment for Paget disease of the nipple is a combination of surgery to remove the affected tissue, followed by radiation therapy or chemotherapy to eliminate any remaining cancer cells. Early detection and treatment of Paget disease of the nipple can significantly improve the prognosis for patients.

Table of Contents:

#️⃣  Coding Considerations

The equivalent SNOMED CT code for the ICD-11 code 2E65.5 (Paget disease of nipple) is 73008002. This SNOMED CT code specifically refers to the condition of Paget’s disease of the nipple, which is a rare form of breast cancer that affects the skin of the nipple and areola. The code 73008002 includes both the diagnosis of Paget’s disease of the nipple and the associated treatment options available for this condition. By using the SNOMED CT code 73008002, healthcare professionals can accurately document and track the presence of Paget’s disease of the nipple in their patients, ensuring appropriate management and care for individuals with this rare form of breast cancer.

In the United States, ICD-11 is not yet in use. The U.S. is currently using ICD-10-CM (Clinical Modification), which has been adapted from the WHO’s ICD-10 to better suit the American healthcare system’s requirements for billing and clinical purposes. The Centers for Medicare and Medicaid Services (CMS) have not yet set a specific date for the transition to ICD-11.

The situation in Europe varies by country. Some European nations are considering the adoption of ICD-11 or are in various stages of planning and pilot studies. However, as with the U.S., full implementation may take several years due to similar requirements for system updates and training.

🔎  Symptoms

Symptoms of 2E65.5 (Paget disease of nipple) may manifest in a variety of ways. Patients with this condition commonly experience persistent itching, redness, or scaling of the nipple and areola. These early symptoms may be mistaken for common dermatological conditions, delaying diagnosis and treatment.

As Paget disease of the nipple progresses, individuals may notice crusting or ulceration of the nipple, discharge, or inversion of the nipple. These symptoms are more indicative of an underlying breast malignancy. It is important for individuals experiencing these symptoms to seek prompt medical evaluation and screening for breast cancer.

In some cases, patients may also report a lump in the breast or axilla. This can be concerning as it may be a sign of an additional primary breast cancer or metastasis. Prompt evaluation with clinical breast exam, imaging studies, and possibly biopsy is crucial in determining the extent of disease and developing an appropriate treatment plan.

🩺  Diagnosis

Diagnosis of Paget disease of the nipple (2E65.5) typically involves a combination of clinical evaluation, imaging studies, and histological examination.

Initially, a physical examination may reveal characteristic signs such as nipple crusting, scaling, or ulceration. The presence of a palpable mass or enlarged lymph nodes in the axilla may also be noted.

Imaging studies such as mammography or breast ultrasound can provide further information on the extent of the disease and identify any underlying breast tissue abnormalities. These can help in staging the disease and guiding treatment planning.

Histological examination, usually through a biopsy of the affected nipple tissue, is the definitive method of confirming a diagnosis of Paget disease. Examination of the tissue under a microscope can reveal the presence of Paget cells, which are large malignant-appearing cells characteristic of the disease. Other tests may be performed to determine the hormone receptor status of the tumor and guide treatment decisions.

💊  Treatment & Recovery

Treatment for Paget disease of the nipple (2E65.5) typically involves a combination of surgical and non-surgical approaches. The main goal of treatment is to remove the affected tissue and prevent the spread of cancerous cells. Surgery is often the primary treatment option, with options including nipple-sparing mastectomy or lumpectomy.

In cases where surgery is not sufficient, additional treatments may be recommended. These may include radiation therapy to target any remaining cancer cells, as well as hormone therapy or chemotherapy to destroy cancer cells and prevent recurrence. The specific treatment plan will depend on the individual patient’s situation, including the extent of the disease and overall health.

Following treatment for Paget disease of the nipple, recovery is an important part of the process. Patients may experience physical and emotional changes as a result of treatment, and support from healthcare providers, counselors, and support groups can be beneficial. Regular follow-up appointments will be necessary to monitor for any signs of recurrence or complications, and to ensure the best possible outcome for the patient.

🌎  Prevalence & Risk

In the United States, the prevalence of Paget disease of the nipple, represented by the ICD-10 code 2E65.5, is estimated to be relatively rare. It is believed to account for less than 5% of all cases of breast cancer diagnosed in women. The disease predominantly affects women over the age of 50, with the majority of cases occurring in postmenopausal women.

In Europe, the prevalence of Paget disease of the nipple is also considered to be relatively low. The exact prevalence rates vary by country, but overall, it is believed to account for a small percentage of all breast cancer cases diagnosed in the region. Like in the United States, the disease predominantly affects older women, particularly those over the age of 50.

In Asia, the prevalence of Paget disease of the nipple is even lower compared to Western countries. The disease is relatively rare in this region, with only a small number of cases reported each year. The exact prevalence rates vary by country, but overall, it is considered to be a rare form of breast cancer in Asia.

In Africa, the prevalence of Paget disease of the nipple is not well-documented. Limited research has been conducted on the disease in African populations, and as a result, there is a lack of data on its prevalence in the region. Further studies are needed to better understand the prevalence and impact of Paget disease of the nipple in African populations.

😷  Prevention

The most effective method of preventing Paget disease of the nipple (2E65.5) is regular breast self-examination. By becoming familiar with the look and feel of one’s breasts, individuals can more easily identify any changes or abnormalities that may indicate the presence of Paget disease. It is recommended that women perform monthly self-exams and report any concerning findings to their healthcare provider promptly.

Early detection plays a crucial role in preventing the progression of Paget disease of the nipple. Therefore, individuals should schedule routine clinical breast exams and mammograms as recommended by their healthcare provider. These screenings can help detect any signs of Paget disease at an early stage when treatment options are more effective.

Maintaining a healthy lifestyle can also contribute to the prevention of Paget disease of the nipple. Eating a balanced diet, engaging in regular physical activity, and avoiding smoking and excessive alcohol consumption are factors that may reduce the risk of developing this condition. Additionally, discussing personal and family medical history with a healthcare provider can help assess individual risk factors for Paget disease and lead to early detection and prevention strategies.

One similar disease to 2E65.5 (Paget disease of nipple) is breast cancer, which is coded as C50 in the International Classification of Diseases (ICD). Breast cancer involves the abnormal growth of cells in the breast tissue, often forming a lump or tumor that can spread to other parts of the body. Like Paget disease of the nipple, breast cancer can manifest with symptoms such as changes in the skin or nipple, including redness, dimpling, or crusting.

Another relevant disease akin to 2E65.5 is eczema (L30.9), a chronic inflammatory skin condition that can cause redness, itching, and flaking of the skin. While eczema typically affects areas such as the hands, arms, and face, it can also appear on the nipples, leading to symptoms similar to those of Paget disease. Eczema on the nipples may result in discomfort, pain, and changes in the appearance of the skin.

Additionally, 2E65.5 is comparable to dermatitis (L30.9), an inflammation of the skin that can be caused by factors such as allergies, irritants, or infections. Dermatitis affecting the nipple can present with redness, swelling, and itching, resembling symptoms of Paget disease. Both conditions may require medical evaluation to determine the underlying cause and appropriate treatment.

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