Kiavash Katrin, Kim Steve, Thompson Andrew D
Department of Pathology, Wayne State University School of Medicine, Detroit Medical Center, Detroit, MI.
Department of Surgery, Wayne State University School of Medicine, Detroit, MI.
Am J Dermatopathol. 2019 Jan;41(1):45-49. doi: 10.1097/DAD.0000000000001208.
Extramammary Paget disease (EMPD) is a rare intraepithelial carcinoma and an uncommon variant of Paget disease affecting areas of the apocrine-rich skin of the perineum, vulva, and less commonly, axilla. Women in their sixth to eighth decades are commonly affected. It is exceedingly rare for EMPD to present on the face, chest, abdomen, or other nonapocrine sites and even more unusual for EMPD to present as a pigmented lesion. The relationship between Paget cells in pigmented extramammary Paget disease (PEMPD) and reactive proliferation and colonization by melanocytes has been poorly explored. The relevance of this rare entity resides in its potential to be misdiagnosed clinically and histopathologically as malignant melanoma in situ. Therefore, application of a panel of immunostains and careful analysis and interpretation of these findings are essential to arrive at the correct diagnosis. We report a new case of PEMPD on a nonapocrine site. The specimen was examined by routine microscopy including hematoxylin and eosin stain as well as immunostains. Histologic examination revealed characteristic features of PEMPD confirmed with immunohistochemical stains.
乳房外佩吉特病（EMPD）是一种罕见的上皮内癌，是佩吉特病的一种不常见变体，累及会阴、外阴等富含顶泌汗腺皮肤区域，腋窝较少见。通常发病于60至80岁的女性。EMPD出现在面部、胸部、腹部或其他非顶泌汗腺部位极为罕见，表现为色素沉着性病变则更为罕见。色素沉着性乳房外佩吉特病（PEMPD）中佩吉特细胞与黑素细胞反应性增殖和定植之间的关系尚未得到充分研究。这种罕见实体的重要性在于其在临床和组织病理学上有被误诊为原位恶性黑色素瘤的可能性。因此，应用一组免疫染色并仔细分析和解读这些结果对于做出正确诊断至关重要。我们报告一例发生于非顶泌汗腺部位的PEMPD新病例。标本通过常规显微镜检查，包括苏木精和伊红染色以及免疫染色。组织学检查显示PEMPD的特征性表现，并经免疫组化染色证实。
