Ectopic Extramammary Paget’s Disease: Case Report and Literature Review

Abstract

Extramammary Paget's disease that occurs in non-apocrine-bearing regions is referred to as ectopic and has been rarely reported. A 62-year-old man presented with a slowly progressive, asymptomatic light-brown plaque on his back. Histopathological examination revealed the presence of large pale cells with prominent nuclei, which proliferated diffusely and focally in the epidermis. Immuno-histochemically the tumour cells were positive for CK7, GCDFP-15, CEA, and p63. Based on these findings, we diagnosed the tumour as ectopic extramammary Paget's disease. We reviewed the English and Japanese literature and found 29 previously reported cases of ectopic extramammary Paget's disease, including our case, with a predominance of occurrence in the Asian population. The germinative milk line is known to be a possible site where extramammary Paget's disease occurs. Like-wise, some germinative apocrine-differentiating cells might exist on the trunk preferentially in Asians. Attention should be paid to the development of ectopic as well as triple or quadruple EMPD in Asians.

Inmunohistoquímica en dermatopatología: revisión de los anticuerpos utilizados con mayor frecuencia (parte i)

“…Los carcinomas espinocelulares cutáneos son habitualmente negativos para esta CK, mientras que los originados en otros epitelios escamosos no queratinizantes, como el cuello uterino, resultan positivos. Es un marcador muy sensible y bastante específico en la identificación de las células neoplásicas de la enfermedad de Paget mamaria y extramamaria 3,12,13 (fig. 6).…”

“…Se ha observado también expresión variable de este marcador en muchas neoplasias ductales y secretoras, con diferenciación ecrina y apocrina ( fig. 14), así como en las células neoplási-cas de la enfermedad de Paget mamaria y extramamaria 12,22 .…”

Primary bilateral extramammary Paget′s disease of the axillae: Another case of this strange disease

“…We decided not to perform any perianal skin biopsy motivated by the high rarity of this co-existence, counting less than 30 cases described in the literature and the majority among Asian men[8], the absolute absence of any signs and symptoms.…”

Extramammary Paget′s disease: Analysis of 17 Chinese cases

“…This male predominance (69-100%) is also reported in other Asian publications from China, Japan, and Korea. [8][9][10] Extramammary Paget's disease also appears to be much more common in Asia than previous reports about the occurrence in Caucasians would indicate. There are various possibilities to explain this difference: (1) Because of the large populations in Asia extramammary Paget's disease is more frequently seen in dermatology departments.…”