TY - JOUR
T1 - Extramammary Paget's disease
T2 - a report of 2 cases and a review of the literature.
AU - Khoo, Joon Joon
AU - Choon, Siew Eng
N1 - Copyright:
This record is sourced from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
PY - 2003/6
Y1 - 2003/6
N2 - Extramammary Paget's disease (EMPD) is a rare disorder and may be found in the vulva, scrotum, penile area, perianal region and the groin. Frequently, it is associated with an underlying regional neoplasm or internal malignancy. We report 2 cases of EMPD; one involving the scrotal area and the other the vulva. Both were elderly patients who presented to the dermatologists with chronic eczematous lesions in the perineum that did not respond to topical treatment. Skin biopsies confirmed extramammary Paget's disease. Investigations for internal malignancies were negative. However, one of the patients defaulted treatment before surgery. The other patient had two excision surgeries with skin grafting to try to achieve tumour free margins. A long term follow-up was planned for him to look for recurrences. These cases emphasise that EMPD can mimic exudative dermatitis and present as a chronic non-healing lesion in the perineum for many years. Clinicians should have a high index of suspicion to pick up the disease early by biopsy. Various immunohistochemical markers not only can help differentiate other histological diagnoses but also help predict the presence of underlying malignancies. Management of EMPD included thorough search for occult or underlying malignancy followed by complete excision surgery with intraoperative frozen sections. Even then, recurrences are high for this disease and long term follow-up is advocated.
AB - Extramammary Paget's disease (EMPD) is a rare disorder and may be found in the vulva, scrotum, penile area, perianal region and the groin. Frequently, it is associated with an underlying regional neoplasm or internal malignancy. We report 2 cases of EMPD; one involving the scrotal area and the other the vulva. Both were elderly patients who presented to the dermatologists with chronic eczematous lesions in the perineum that did not respond to topical treatment. Skin biopsies confirmed extramammary Paget's disease. Investigations for internal malignancies were negative. However, one of the patients defaulted treatment before surgery. The other patient had two excision surgeries with skin grafting to try to achieve tumour free margins. A long term follow-up was planned for him to look for recurrences. These cases emphasise that EMPD can mimic exudative dermatitis and present as a chronic non-healing lesion in the perineum for many years. Clinicians should have a high index of suspicion to pick up the disease early by biopsy. Various immunohistochemical markers not only can help differentiate other histological diagnoses but also help predict the presence of underlying malignancies. Management of EMPD included thorough search for occult or underlying malignancy followed by complete excision surgery with intraoperative frozen sections. Even then, recurrences are high for this disease and long term follow-up is advocated.
UR - http://www.scopus.com/inward/record.url?scp=27244439972&partnerID=8YFLogxK
M3 - Article
C2 - 16196382
AN - SCOPUS:27244439972
SN - 0126-8635
VL - 25
SP - 73
EP - 78
JO - The Malaysian Journal of Pathology
JF - The Malaysian Journal of Pathology
IS - 1
ER -