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皮肤炎性假瘤——是一系列不同疾病吗?

Cutaneous inflammatory pseudotumor--a spectrum of various diseases?

作者信息

El Shabrawi-Caelen Laila, Kerl Katrin, Cerroni Lorenzo, Soyer H Peter, Kerl Helmut

机构信息

Department of Dermatology, Medical University of Graz, Graz, Austria.

出版信息

J Cutan Pathol. 2004 Oct;31(9):605-11. doi: 10.1111/j.0303-6987.2004.00239.x.

DOI:10.1111/j.0303-6987.2004.00239.x
PMID:15330992
Abstract

BACKGROUND

Inflammatory pseudotumor (IPT) also known as inflammatory myofibroblastic tumor (IMT) or plasma cell granuloma (PCG) has been rarely reported in the skin.

METHODS

We describe five patients with cutaneous IPT and present clinicopathologic features along with detailed immunohistochemical analysis including anaplastic lymphoma kinase (ALK)-1.

RESULTS

The patients age ranged from 15 to 89 years with a median of 56 years. All patients presented with solitary, firm, papules and nodules. There was no evidence of constitutional symptoms, local recurrence, or lymph node involvement. Histopathological examination revealed two distinct patterns; one type (n = 3) displayed dense, lymphoplasmacytoid infiltrates containing lymphoplasmacytoid cells and plasma cells with occasional germinal centers and hyalinized collagen bundles but was devoid of a myofibroblastic component. It showed features of tumors previously described as cutaneous PCG. Although an infectious etiology, including Borrelia burgdorferi-specific DNA, could not be demonstrated, we observed many features that overlapped with those of fibrous nodules of acrodermatitis chronica atrophicans. The other pattern (n = 2) revealed spindled myofibroblasts focally arranged in a fascicular pattern, an admixed lymphoplasmacytoid infiltrate set in a background of thickened collagen bundles, findings akin to the conventional type of IMT. The cases with a myofibroblastic component (n = 2) did not show any evidence of ALK-1 reactivity.

CONCLUSIONS

We believe that the term cutaneous IPT subsumes lesions of diverse etiology. Tumors with detectable myofibroblasts represent true cases of IMT. Cutaneous PCG is a discrete disorder biologically distinct from conventional IMT representing a reaction pattern that is also found in disorders, such as spirochete-induced fibroid nodules and localized chronic fibrosing vasculitis.

摘要

背景

炎性假瘤(IPT),也称为炎性肌成纤维细胞瘤(IMT)或浆细胞肉芽肿(PCG),在皮肤中很少见。

方法

我们描述了5例皮肤IPT患者,并展示了临床病理特征以及详细的免疫组化分析,包括间变性淋巴瘤激酶(ALK)-1。

结果

患者年龄从15岁到89岁不等,中位数为56岁。所有患者均表现为孤立、坚实的丘疹和结节。没有全身症状、局部复发或淋巴结受累的证据。组织病理学检查显示两种不同的模式;一种类型(n = 3)表现为密集的淋巴浆细胞样浸润,含有淋巴浆细胞样细胞和浆细胞,偶尔有生发中心和玻璃样变的胶原束,但没有肌成纤维细胞成分。它显示出先前描述为皮肤PCG的肿瘤特征。虽然无法证明包括伯氏疏螺旋体特异性DNA在内的感染病因,但我们观察到许多与慢性萎缩性肢端皮炎纤维结节重叠的特征。另一种模式(n = 2)显示梭形肌成纤维细胞局部呈束状排列,在增厚的胶原束背景中有混合的淋巴浆细胞样浸润,这一发现类似于传统类型的IMT。具有肌成纤维细胞成分的病例(n = 2)未显示任何ALK-1反应性的证据。

结论

我们认为皮肤IPT这一术语涵盖了病因多样的病变。具有可检测到的肌成纤维细胞的肿瘤代表IMT的真正病例。皮肤PCG是一种生物学上与传统IMT不同的离散性疾病,代表一种在诸如螺旋体诱导的纤维瘤结节和局限性慢性纤维化血管炎等疾病中也能发现的反应模式。

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