Histological features of localized scleroderma 'en coup de sabre' : a study of 16 cases
Histological features of localized scleroderma 'en coup de sabre' : a study of 16 cases
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局限性硬皮病“en coup de sabre”的组织学特征：16例研究
DOI：
10.1111/jdv.12280
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发表时间：
2014
期刊：
影响因子：
--
通讯作者：
Taniguchi Takashi
中科院分区：
文献类型：
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作者：
Okamoto Y.;Nakai T.;Ando M.;Ueda K. and Kojima N.;中井剛;Yumeka Hirano;中井 剛;Yumeka Hirano;中井剛;Yumeka Hirano and Shigeru Otsubo;Yumeka Hirano and Shigeru Otsubo;中井剛;中井剛;Yumeka Hirano and Shigeru Otsubo;Yumeka Hirano;Shigeru Otsubo and Yumeka Hirano;Yumeka Hirano;Yumeka Hirano and Shigeru Otsubo;Taniguchi Takashi
背景 局限性硬皮病的早期病变的组织学特征是网状真皮中血管周围淋巴细胞浸润和内皮细胞肿胀。然而，关于局限性硬皮病除这些发现之外的组织学特征的信息很少。目的由于ECDS是局限性硬皮病的一个亚型，其临床表现相对统一，我们重点关注该疾病亚型并评估其组织病理学特征。方法根据临床和组织学表现，对16例ECDS患者进行回顾性评估。结果无论临床表现如何，均发现空泡变性。在所有 ECDS 患者中。重要的是，角质形成细胞坏死仅限于早期和活动性 ECDS 病变。与晚期ECDS患者（病程<3年）相比，早期ECDS患者（病程<3年）中度至重度血管周围和/或阑尾周围淋巴细胞浸润和毛囊上皮空泡变化更为明显，而表皮萎缩则较晚期ECDS患者（病程≥6年）少见。结论真皮表皮交界处的空泡变性是常见的组织学特征ECDS 和血管周围和/或阑尾周围淋巴细胞浸润和滤泡上皮空泡变性是早期 ECDS 的特征，进一步支持了一个经典观点，即通过免疫监视消除突变的表皮细胞会导致局部硬皮病的组织损伤和由此产生的纤维化。
BackgroundEarly lesions of localized scleroderma are histologically characterized by perivascular lymphocytic infiltrate in the reticular dermis and swollen endothelial cells. However, there have been few information regarding histological features other than these findings in localized scleroderma.ObjectiveSinceen coup de sabre(ECDS) is a certain subset of localized scleroderma with a relatively uniform clinical manifestation, we focused on this disease subset and evaluated its histopathological features.MethodsA total of 16 patients with ECDS were retrospectively evaluated on the basis of clinical and histological findings.ResultsRegardless of clinical manifestations, vacuolar degeneration was found in all of the ECDS patients. Importantly, keratinocyte necroses were restricted to early and active ECDS lesions. In early ECDS patients (disease duration of <3 years), moderate to severe perivascular and/or periappendageal lymphocytic infiltrate and vacuolar changes in follicular epithelium were more prominent, whereas epidermal atrophy was less frequently observed, than in late ECDS patients (disease duration of ≥6 years).ConclusionVacuolar degeneration at the dermoepidermal junction is a common histological feature in ECDS and perivascular and/or periappendageal lymphocytic infiltrate and vacuolar degeneration of follicular epithelium are characteristic especially in early ECDS, further supporting a canonical idea that the elimination of mutated epidermal cells by immune surveillance contributes to tissue damage and resultant fibrosis in localized scleroderma.