Granulomatous slack skin: clonal rearrangement of the T-cell receptor beta gene is evidence for the lymphoproliferative nature of a cutaneous elastolytic disorder.
Granulomatous slack skin: clonal rearrangement of the T-cell receptor beta gene is evidence for the lymphoproliferative nature of a cutaneous elastolytic disorder.
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肉芽肿性松弛皮肤：T 细胞受体 β 基因的克隆重排是皮肤弹力溶解障碍的淋巴增殖性质的证据。
DOI：
10.1111/1523-1747.ep12470557
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发表时间：
1987
期刊：
影响因子：
--
通讯作者：
Parslow,TG
中科院分区：
文献类型：
--
作者：
LeBoit,PE;Beckstead,JH;Bond,B;Epstein,WL;Frieden,IJ;Parslow,TG
肉芽肿性松弛皮肤（GSS）的特征是具有肉芽肿组织学的笨重，红斑性皮肤褶皱的缓慢演变，并表现出真皮弹性组织的破坏。一些病例被认为与何杰金氏病有关，并且与蕈样真菌病的组织学相似也被注意到。我们检查了3例GSS的组织，以确定这种情况是炎症性的还是淋巴增生性的。我们发现了一个异常的，单形的t辅助细胞免疫表型，并且在所有3例中，t细胞受体β基因克隆重排。我们得出结论，GSS是一种惰性皮肤t细胞淋巴瘤，与肉芽肿性炎症相关，介导弹性溶解，产生独特的临床表现。
Granulomatous slack skin (GSS) is characterized by the slow evolution of bulky, erythematous skin folds that have a granulomatous histology, and show destruction of dermal elastic tissue. Several cases have been putatively associated with Hodgkin's disease, and histologic similarities to mycosis fungoides have also been noted. We examined tissue from 3 cases of GSS to determine whether the condition was inflammatory or lymphoproliferative in nature. We found an abnormal, monomorphous T-helper cell immunophenotype, and in all 3 cases, Clonal rearrangement of the T-cell receptor β gene. We conclude that GSS is an indolent cutaneous T-cell lymphoma associated with granulomatous inflammation that mediates elastolysis, producing a distinctive clinical appearance.