Extramammary Paget's Disease (EMPD) is a rare skin condition that primarily affects the genital and perianal areas, often presenting in older adults. It is characterized by malignant changes in the skin and can sometimes be associated with underlying internal malignancies. Recent research has focused on improving diagnostic methods, treatment options, and understanding the disease's behavior, particularly regarding recurrence rates and the effectiveness of various therapies.
Recent Research
One significant advancement in EMPD research is the development of evidence-based clinical practice guidelines. A systematic review of the literature has led to recommendations emphasizing the importance of multiple skin biopsies for accurate diagnosis and the necessity of screening for associated malignancies. The guidelines suggest that surgical resection is the preferred treatment for invasive EMPD, while non-invasive cases may benefit from a range of nonsurgical treatments, including topical therapies and radiotherapy[1].
Imiquimod, a topical immune response modifier, has emerged as a promising treatment for non-invasive forms of vulvar Paget disease, which is closely related to EMPD. Recent studies have shown that imiquimod can lead to significant clinical responses, with a notable percentage of patients achieving complete remission. However, side effects such as fatigue and headaches were common, and some patients required adjustments to their treatment regimen[3][4]. This highlights the need for ongoing evaluation of patient quality of life alongside treatment efficacy.
Another area of focus has been the comparison of surgical techniques for treating EMPD. A systematic review and meta-analysis indicated that Mohs micrographic surgery (MMS) results in lower local recurrence rates compared to wide local excision (WLE). Patients treated with MMS had a significantly reduced risk of recurrence, suggesting that this technique may be more effective for managing EMPD[5]. This finding is crucial for guiding treatment decisions and improving long-term outcomes for patients.
Additionally, research into the immunohistochemical expression of TRPS1 has shown promise in differentiating EMPD from other similar skin conditions. The presence of TRPS1 was found in a significant proportion of EMPD cases, which could aid in accurate diagnosis and treatment planning[2].
Technical Terms
Extramammary Paget's Disease (EMPD): A rare skin condition characterized by malignant changes in the skin, typically occurring in the genital and perianal areas.
Imiquimod: A topical medication that stimulates the immune system to fight certain skin conditions, including some forms of skin cancer.
Mohs Micrographic Surgery (MMS): A surgical technique used to treat skin cancer, which involves removing the cancerous tissue layer by layer and examining it microscopically for cancer cells.
Wide Local Excision (WLE): A surgical procedure that involves removing the tumor along with a margin of healthy tissue surrounding it.
References
Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease
Immunohistochemical expression of TRPS1 in mammary Paget disease, extramammary Paget disease, and their close histopathologic mimics
The Paget Trial: topical 5% imiquimod cream for noninvasive vulvar Paget disease
Vulvar Paget's disease: outcomes of 51 patients treated with imiquimod cream
Local Recurrence Rates of Extramammary Paget Disease Are Lower After Mohs Micrographic Surgery Compared With Wide Local Excision: A Systematic Review and Meta-Analysis
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