Overview
Extramammary Paget disease (EMPD) is a rare malignancy typically arising in apocrine gland-bearing areas such as the genital, perianal, and axillary regions 1.Diagnosis
Clinical Presentation: Typically involves persistent, scaly, eczematous patches in affected areas 1.
Biopsy: Essential for diagnosis; mapping biopsy recommended for extent evaluation 1.
Mucin Staining: Rapid mucicarmine staining technique on frozen sections can aid in confirming mucin production, crucial for diagnosis 2.Management
Surgical Excision: Primary treatment, often requiring wide local excision with clear margins 1.
Sentinel Lymph Node Biopsy: Considered in cases with high suspicion of lymph node involvement 1.
Adjuvant Therapy: Not routinely recommended without specific risk factors or advanced disease stages 1.Special Populations
No Specific Guidance: Abstracts do not provide detailed recommendations for pregnancy, pediatrics, elderly, or comorbid conditions 12.Key Recommendations
Utilize mapping biopsy for accurate assessment of disease extent in EMPD management (Evidence: Moderate) 1.
Consider sentinel lymph node biopsy in cases where lymph node metastasis is suspected (Evidence: Moderate) 1.
Employ rapid mucicarmine staining technique on frozen sections to aid in rapid diagnosis (Evidence: Weak) 2.References
1 Matsushita S, Kajihara I, Tsutsui K, Hirai I, Maekawa T, Maeda T et al.. Japanese Dermatological Association Guidelines: Clinical Questions of Guidelines for Extramammary Paget Disease 2025. The Journal of dermatology 2025. link
2 Soans S, Galindo LM, Garcia FU. Mucin stain on frozen sections: a rapid 3-minute method. Archives of pathology & laboratory medicine 1999. link