THE CLINICAL picture that Paget1 described in 1874 as a disease of the mammary areola preceding cancer of the mammary gland was soon known to have not only characteristic clinical features but also a peculiar microscopic picture, which entitled it to be classified in a separate group of cutaneous epitheliomatoses.
When it was observed that similar pathologic changes could be diagnosed in regions of the skin other than the mammary areola, there began a prolonged controversy as to the precise nature and site of origin of Paget's disease and its peculiar cells (to which the term Paget's cells has been applied), which still persists.
The disease is rare. The nipple is the commonest site; women are more commonly affected than men. However, among the 33 published cases of extramammary Paget's disease, 14 have been described as affecting the external genital organs; only 8 cases have been accepted, as far
CASPER WA. PAGET'S DISEASE OF THE VULVA. Arch Derm Syphilol. 1948;57(4):668–678. doi:10.1001/archderm.1948.01520170066009
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