Extramammary Paget's Disease Quiz

What is Extramammary Paget's Disease?

Skin rashes can be VERY confusing. Most are due to contact with something irritating and are easily treated with over the counter creams. However, there are some that need to be seen by a physician and possibly biopsied. One of these is Extramammary Paget's disease (EMPD). It is a scaly, raised red to pink and grey rash that occurs most commonly where the skin is folded over such as the armpits, the genital areas, or the groin in men or women. EMPD is often ignored or diagnosed late due to its innocent appearance. It is important to see a physician if you have a rash in these areas that does not go away as it may be cancerous. Multiple treatments are available and patients do well when it is caught early.

Typical Symptoms of Extramammary Paget's Disease

• Black/blue/brown spots or moles, on the skin and lining of the mouth
• White patches on the skin
• Itchy skin
• Skin lumps
• Dry and flaky skin
• Scab
• Pain or discomfort in the genital area
• Redness, bumps, or irritation on the skin

Diagnostic Questions for Extramammary Paget's Disease

Treatment of Extramammary Paget's Disease

The standard treatment is surgical removal of the cancer. Radiotherapy, chemotherapy, and targeted therapies may also be used depending on the exact nature of the underlying tumor if one is found. Surgical resection is the preferred treatment, however some lesions may not be easily removed surgically and may require radiation therapy or chemotherapy. There are also surface treatments such as lasers or photodynamic therapy that may be used. Close monitoring after successful treatment should be done for at least 5 years.

Symptoms Related to Extramammary Paget's Disease

• Moles on the skin and lining of the mouth
• Lighter patches of skin
• Itchy skin
• Lumps under the skin
• Scaling skin

Diseases Related to Extramammary Paget's Disease

• Lichen Sclerosus et Atrophicus
• Genital Rashes
• Crab Louse
• Epidermoid Cyst
• Trichomoniasis

References

• Kibbi N, Owen JL, Worley B, Wang JX, Harikumar V, Downing MB, Aasi SZ, Aung PP, Barker CA, Bolotin D, Bordeaux JS, Cartee TV, Chandra S, Cho NL, Choi JN, Chung KY, Cliby WA, Dorigo O, Eisen DB, Fujisawa Y, Golda N, Halfdanarson TR, Iavazzo C, Jiang SIB, Kanitakis J, Khan A, Kim JYS, Kuzel TM, Lawrence N, Leitao MM, MacLean AB, Maher IA, Mittal BB, Nehal KS, Ozog DM, Pettaway CA, Ross JS, Rossi AM, Servaes S, Solomon MJ, Thomas VD, Tolia M, Voelzke BB, Waldman A, Wong MK, Zhou Y, Arai N, Brackett A, Ibrahim SA, Kang BY, Poon E, Alam M (Year). Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease. JAMA oncology.

  https://pubmed.ncbi.nlm.nih.gov/35050310/

• Shah RR, Shah K, Wilson BN, Tchack M, Busam KJ, Moy A, Leitao MM, Cordova M, Neumann NM, Smogorzewski J, Nguyen KA, Hosein S, Dafinone M, Schwartz RA, Rossi A (Year). Extramammary Paget disease. Part I. epidemiology, pathogenesis, clinical features, and diagnosis. Journal of the American Academy of Dermatology.

  https://pubmed.ncbi.nlm.nih.gov/38704032/

• Shah RR, Shah K, Wilson BN, Leitao MM, Smogorzewski J, Nguyen KA, Crane C, Funt SA, Hosein S, Dafinone M, Rossi A (Year). Extramammary Paget disease. Part II. Evidence-based approach to management. Journal of the American Academy of Dermatology.

  https://pubmed.ncbi.nlm.nih.gov/38588817/