Pranteda G, Gueli N, Bottoni U, Pranteda G, Calvieri S
Institute of Dermatology, University of Rome La Sapienza, Italy.
J Exp Clin Cancer Res. 1998 Dec;17(4):519-22.
Paget's disease is usually associated with an underlying adenocarcinoma of the breast. The initial manifestation is an eczematous or psoriasiform lesion of the nipple, soon extending to the mammary areola and then to the surrounding skin. The histology of the lesions is characterized by the presence, within the epidermal layer, of the so-called Paget's cells, i.e. large cells with vesicular nucleus and clear cytoplasm. The authors report a recent case of Paget's disease of the breast in a 75-year-old woman, unusual both for clinical course and observed histology. As for the clinico-evolutive aspects, although in the case observed the initial skin lesions appeared 20 years before, the different diagnostic procedures repeatedly performed showed no evidence in this patient of an underlying adenocarcinoma. The histologic aspect, on the other hand, was peculiar since the typical characteristics of an acantholytic disease were evident. The presence of intraepidermal cleavages with lost, at times, of the normal contacts among the cells of the Malpighian layer has been described only once in Paget's disease. This fact caused some diagnostic difficulties: however, the typical finding of the Paget cells, their positivity to histochemical methods such as cytokeratin and acid phosphatase allowed the diagnosis. The authors, at last, evaluate the need of a surgical therapy in Paget's disease of the breast without an underlying adenocarcinoma.
佩吉特病通常与潜在的乳腺腺癌相关。最初的表现是乳头的湿疹样或银屑病样病变，很快蔓延至乳晕，然后累及周围皮肤。病变的组织学特征是在表皮层内存在所谓的佩吉特细胞，即具有泡状核和透明细胞质的大细胞。作者报告了一例75岁女性的乳腺佩吉特病，其临床病程和观察到的组织学表现均不寻常。至于临床演变方面，尽管在观察到的病例中最初的皮肤病变出现在20年前，但反复进行的不同诊断程序均未显示该患者存在潜在腺癌的证据。另一方面，组织学表现很特殊，因为棘层松解性疾病的典型特征很明显。表皮内裂隙的存在，有时马尔皮基层细胞之间正常联系丧失，在佩吉特病中仅被描述过一次。这一事实造成了一些诊断困难：然而，佩吉特细胞的典型发现，它们对细胞角蛋白和酸性磷酸酶等组织化学方法呈阳性，使得能够做出诊断。最后，作者评估了在无潜在腺癌的乳腺佩吉特病中进行手术治疗的必要性。
