Vulva paget disease

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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. 10 of patients are asymptomatic resulting in delayed diagnosis.   pagets disease of the vulva is a very rare form of pagets disease that arises from a location outside the breast (extramammary site). In addition to the vulva, extramammary pagets disease (empd) can also arise in the groin (penis and scrotum) and perineum region). Pagets disease of the vulva is an unusual kind of skin cancer that arises from glandular cells. This disease appears as a red, velvety area with white islands of tissue on the vulva. At times it may be pink, and occasionally there are moist, oozing ulcerations that bleed easily. Pagets disease can arise in the breast (mammary paget disease mpd) or in other locations (extramammary paget disease empd) such as the anogenital skin in females (paget disease of the vulva pdv). Pdv is an uncommon pathologic condition 18 accounting for from 1 to 2 of all tumors of the female genital area. Authors m van der linden 1 , k a p meeuwis 2 , j bulten 3 , t bosse 4 , m i e van poelgeest 5 , j a de hullu 6 affiliations 1 department of obstetrics & gynaecology, radboud.   in 1901, paget disease of the vulva was described for the first time by a french dermatologist, william dubreuilh (dubreuilh, 1901). Mammary and extramammary paget disease are characterized by the presence of intraepithelial mucin-producing neoplastic cells known as paget cells.

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