H10 Extramammary Paget disease: from the 19th century to now – where ‘Paget’ meets the scrotum and beyond

Abstract Sir James Paget (1814–1899), born in Great Yarmouth, Norfolk, and a surgeon at St Bartholomew’s Hospital in London, first described Paget disease of the breast in 1874. He identified 15 women with chronic nipple lesions described as ‘eczematous ulcerative or vesicular lesions with clear yellowish exudate’. All these cases later developed into malignant lesions and this condition was subsequently termed mammary Paget disease. In 1887, Henry Radcliffe Crocker (1846–1909) documented the first case of what is today known as extramammary Paget disease (EMPD). Crocker was a dermatologist at University College Hospital. He was a founding member of the Dermatological Society of London in 1899 and president of the Dermatological Society of Great Britain and Northern Ireland. In ‘Paget’s disease, affecting the scrotum and penis’, published 1889, Crocker detailed a 60-year-old man presenting with an ‘oozing, well-defined area of superficial ulceration’ on the front, left side of the scrotum and under the surface of the penis. He described similarities to eczema but that the lesion was much deeper and well defined. Many treatments and remedies had no effect. Two nodules later appeared in the affected area, suggesting malignancy. This case was presented by Crocker at the Dermatological Society of London, where members identified similarities to mammary Paget disease. Sir James Paget reviewed the patient and agreed with the similarities, suggesting excision of the affected area. Cancerous cells were seen on microscopic investigation of the nodules. Crocker described the sweat coils and ducts forming a cancerous structure, in keeping with the known pathophysiology of EMPD primarily affecting apocrine-rich areas. Darier and Coulillaud reported a patient with perianal manifestation of Paget disease in 1893. These cases identified this rare but significant skin condition associated with underlying malignancy. Today, EMPD represents approximately 6.5% of all cutaneous Paget disease. The management of EMPD has changed little since it was first described in the 19th century, with identification and treatment of an associated malignancy and surgery remaining the mainstay of treatment.