文献简介

出版社:JAMA

作  者:AshleeM.Margheim,BSN;EmilyC.McKenzie,MD

编  号:

关键字:Paget样网状细胞增多症

年  份:2020   点击量:239

文献摘要 全文翻译

A preschool-age girl presented for evaluation of an asymptomatic plaque on the right lower extremity that had been present for 1 year. Prior treatments included triamcinolone ointment and frankincense with no improvement. A potassium hydroxide preparation had negative results for fungal elements. Her medical history and family history were unremarkable. An examination found a 1-cm nummular plaque with centralized micaceous scale and a smooth erythematous border on the right lateral lower leg (Figure, A). A tangential shave biopsy was performed to characterize the lesion. Histopathological testing revealed an epidermis featuring elongated rete ridges, keratinocyte enlargement, and a few Civatte bodies, with overlying parakeratotic crust and neutrophilic cell debris. Marked epidermotropism of atypical lymphocytes with irregular nuclear contours and halos, both singly and in small collections (Pautrier microabscesses) were also observed (Figure, B and C). Also, lymphocytes were present along the epidermal side of the dermoepidermal junction. Periodic acid–Schiff stained sections failed to show fungal or yeast elements. Immunohisto-chemical staining was markedly positive for CD3, CD5, and CD8(Figure, D); CD4 reactivity was less robust and mainly restricted to dermal lymphocytes. Also, CD20 was present in 5% of dermal lymphocytes, and CD30 showed variable staining of 5% of lymphocytes.

患者学龄前女童,因评估1年前右下肢出现的无症状斑块而就诊。先前治疗包括曲安奈德软膏和乳香,但未改善。氢氧化钾制剂检测结果显示真菌阴性。患者无显著病史和家族史。检查发现一1cm环形斑块,伴中心云母状鳞屑,右侧小腿可见光滑红斑边界(图A)。行正刮取活检以表征皮损。组织病理学检查显示表皮具有细长网状脊,角质形成细胞增大和可见一些Civatte小体,上覆角化不全痂皮和嗜中性细胞碎片。还观察到具有不规则核轮廓和光晕的非典型淋巴细胞显著亲表皮性,无论是单独的还是少量聚集细胞(Pautrier微脓肿)(图B和C)。此外,真皮表皮连接处的表皮侧存在淋巴细胞。高碘酸-希夫(Schiff)染色切片未见真菌或酵母元素。免疫组化染色显示CD3,CD5和CD8呈强阳性(图D)。CD4反应活性较弱,主要局限于皮肤淋巴细胞。同样,5%的真皮淋巴细胞中存在CD20,而可变染色示5%淋巴细胞存在CD30。