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皮肤B细胞淋巴增殖性疾病中出现的皮肤松弛症。

Anetoderma arising in cutaneous B-cell lymphoproliferative disease.

作者信息

Kasper R C, Wood G S, Nihal M, LeBoit P E

机构信息

Department of Pathology, University of California, San Francisco 94115, USA.

出版信息

Am J Dermatopathol. 2001 Apr;23(2):124-32. doi: 10.1097/00000372-200104000-00007.

DOI:10.1097/00000372-200104000-00007
PMID:11285407
Abstract

Anetoderma is circumscribed atrophy of the skin due to a localized deficiency in elastic tissue. It can follow inflammatory skin diseases of several types, and occasionally is present in the skin around neoplasms. There are a few reports of anetoderma in the lesional skin of cutaneous lymphoma. We report on two patients who presented with multiple lesions of anetoderma and who later proved to have low-grade cutaneous B-cell lymphomas. One patient (Patient 1) is a 39-year-old man and the other patient is a 26-year-old woman who is a renal transplant recipient (Patient 2). Some biopsy specimens from the anetodermic skin of Patient 1 appeared to show an urticarial reaction, although plasma cells were present. A large nodule showed lymphoid follicles surrounded by plasmacytoid lymphocytes, with loss of elastic tissue in the adjacent dermis. The plasmacytoid cells stained overwhelmingly for lambda light chain, and staining of the urticarial lesions from this patient also showed a marked majority of lambda positive cells. Immunoglobulin heavy chain gene (IgH) rearrangements showed a dominant clonal pattern in the nodular lesion. We classified the disease in Patient 1 as marginal zone lymphoma and the disease in Patient 2 as a post-transplant lymphoproliferative disorder. Because of the intimate association of anetoderma and cutaneous B-cell lymphoproliferative disorders in these two patients, it seems possible that anetoderma could result from either a local effect of the neoplastic cells or associated inflammatory cells, especially neutrophils as in Case 1. The infiltrates of Case 1 had many interstitial neutrophils and only a few clonal plasmacytoid lymphocytes, indicating that this presentation of B-cell lymphoma can be a diagnostic pitfall. Given these two cases and similar ones in the literature, biopsy of lesional skin in anetoderma should be performed to ensure that lymphomatous infiltrates are not present. Even if plasma cells are sparse, studies to detect clonality are appropriate. Cutaneous B-cell lymphoma can be added to the list of associations of elastolysis and cutaneous lymphoma, which includes granulomatous slack skin (T-cell lymphoma) and cutis laxa (myeloma).

摘要

皮肤弹性纤维松解症是由于弹性组织局部缺乏导致的局限性皮肤萎缩。它可继发于多种类型的炎症性皮肤病,偶尔也出现在肿瘤周围的皮肤。有一些关于皮肤淋巴瘤皮损处出现皮肤弹性纤维松解症的报道。我们报告了两名出现多发性皮肤弹性纤维松解症皮损的患者,后来证实患有低度皮肤B细胞淋巴瘤。一名患者(患者1)是一名39岁男性,另一名患者是一名26岁女性肾移植受者(患者2)。患者1皮肤弹性纤维松解症部位的一些活检标本似乎显示有荨麻疹反应,尽管存在浆细胞。一个大结节显示有被浆细胞样淋巴细胞包围的淋巴滤泡,相邻真皮中的弹性组织缺失。浆细胞样细胞绝大多数为λ轻链染色阳性,该患者荨麻疹皮损的染色也显示绝大多数细胞为λ阳性。免疫球蛋白重链基因(IgH)重排在结节性病变中显示出优势克隆模式。我们将患者1的疾病分类为边缘区淋巴瘤,将患者2的疾病分类为移植后淋巴细胞增生性疾病。由于这两名患者中皮肤弹性纤维松解症与皮肤B细胞淋巴增生性疾病密切相关,皮肤弹性纤维松解症似乎可能是肿瘤细胞或相关炎症细胞的局部作用所致,尤其是如病例1中的中性粒细胞。病例1中的浸润有许多间质中性粒细胞,只有少数克隆性浆细胞样淋巴细胞,表明这种B细胞淋巴瘤的表现可能是一个诊断陷阱。鉴于这两个病例以及文献中类似的病例,应对皮肤弹性纤维松解症的皮损进行活检,以确保不存在淋巴瘤浸润。即使浆细胞稀少,进行检测克隆性的研究也是合适的。皮肤B细胞淋巴瘤可被添加到弹性组织松解与皮肤淋巴瘤的关联列表中,其中包括肉芽肿性皮肤松弛症(T细胞淋巴瘤)和皮肤松弛症(骨髓瘤)。

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引用本文的文献

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A Literature Revision in Primary Cutaneous B-cell Lymphoma.原发性皮肤B细胞淋巴瘤的文献综述
Indian J Dermatol. 2017 Mar-Apr;62(2):146-157. doi: 10.4103/ijd.IJD_74_17.
2
Cutaneous primary B-cell lymphomas: from diagnosis to treatment.皮肤原发性B细胞淋巴瘤:从诊断到治疗
An Bras Dermatol. 2015 Sep-Oct;90(5):687-706. doi: 10.1590/abd1806-4841.20153638.