It is itchy and sometimes pain can be associated with the affected area. The lesion is clearly separated from normal skin in most cases, and sometimes scattered areas of white scale can be present, giving a "strawberries and cream" appearance.Involvement may be extensive including the perianal region, genitocrural, and inguinal folds. Clinical examination should determine the presence of periurethral and perianal lesions. In these cases an involvement of the skin by a noncutaneous internal neoplasm may occur. Pathophysiology
EMPD occurs due to an invasion of the epidermis by Paget cells. The cause of the disease is still under debate with recent research indicating that the disease may be associated with Toker cells. Disease of the vulva
Originates from local organs such as the Bartholin gland, the urethra, or the rectum. Predilection towards postmenopausal women. Metastatic disease
Metastasis of Paget cells from the epidermis to distant regions is a multistep process that involves:

Invasion of local lymph nodes and venous system
Movement out from lymph nodes and venous system
Proliferation at new siteProtein molecules HER2 and mTOR expressed in Paget cells are responsible for providing characteristics of proliferation and survival. Diagnosis
Due to the rarity of EMPD and lack of clinical knowledge, the disease is not very commonly diagnosed. Patients are often misdiagnosed with eczema or dermatitis and a delay of 2 years is expected from the onset of symptoms before a definitive diagnosis has been reached.It is important to include that the lesion is associated with another cancer. A biopsy will establish the diagnosis.
Extramammary Pagets Disease (EMPD) is a rare and slow-growing malignancy which occurs within the epithelium and accounts for 6.5% of all Pagets disease. The clinical presentation of this disease is similar to the characteristics of mammary Pagets disease (MPD). However, unlike MPD, which occurs in large lactiferous ducts and then extends into the epidermis, EMPD originates in glandular regions rich in apocrine secretions outside the mammary glands. EMPD incidence is increasing by 3.2% every year, affecting hormonally-targeted tissues such as the vulva and scrotum. In women, 81.3% of EMPD cases are related to the vulva, while for men, 43.2% of the manifestations present at the scrotum.The disease can be classified as being either primary or secondary depending on the presence or absence of associated malignancies. EMPD presents with typical symptoms such as scaly, erythematous, eczematous lesions accompanied by itchiness. In addition to this, 10% of patients are often asymptomatic. As a consequence, EMPD has high rates of misdiagnoses and delayed diagnoses. There are a variety of treatment options available, but most are unsuccessful. If caught early and treated, prognosis is generally good. Presentation
Patients with EMPD present with typical symptoms, similar to MPD, such as severe itchiness (also called pruritus), rash, plaque formation, burning sensation, pain and tenderness. These symptoms are often confused for dermatitis or eczema. 10% of patients are asymptomatic resulting in delayed diagnosis. In rare cases bleeding can also be seen. Disease of the vulva
Vulvar Pagets disease affect women and presents as erythematous (red), eczematous lesions.