Cutaneous lymphomatoid granulomatosis - Correlation of clinical and biologic features
Cutaneous lymphomatoid granulomatosis - Correlation of clinical and biologic features
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DOI：
10.1097/00000478-200109000-00001
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发表时间：
2001-09-01
影响因子：
5.6
通讯作者：
Jaffe, ES
中科院分区：
文献类型：
--
作者：
Beaty, MW;Toro, J;Jaffe, ES
淋巴瘤样肉芽肿病(LYG)是一种罕见的血管中心性和血管破坏性EB病毒相关性B细胞淋巴增生性疾病(EBV-BLPD)，从缓慢的过程到侵袭性的大细胞淋巴瘤变化很大。皮肤是LYG最常见的肺外器官。我们用免疫组织化学、EBV原位杂交和聚合酶链式反应检测抗原受体基因重排(IgH和TCR)对20例已知肺LYG患者的32个皮肤病变进行了研究，以更好地确定皮肤病变的临床病理谱和发病机制。我们描述了两种不同的皮肤受累模式。17例患者(85%)出现多发性红斑性皮肤丘疹和/或皮下结节，伴有或不伴有溃疡。这些病变显示明显的血管中心性淋巴组织细胞浸润物，主要由CD4阳性的T细胞组成，很容易累及皮下组织，并表现出血管破坏、坏死和细胞学异型性。5例结节中检出EB病毒阳性B细胞，2例用聚合酶链式反应检测到克隆性免疫球蛋白重链基因重排。3例患者(15%)出现多发性硬化、红斑至白色斑块。所有病例的斑块病变EBV和克隆性IgH基因重排均为阴性。整个疾病的临床过程是不同的，从未经治疗的自发消退(1/13；7%)，化疗/免疫调节治疗(8/13，62%)，以及进展(4/13，31%)不等。皮肤LYG的临床和组织病理学特征非常不同。然而，大多数(85%)的皮肤病变在一定程度上反映了肺部的LYG，尽管EBV+细胞较少被发现。这一亚组病例显示血管破坏的组织病理学三联征，伴有相关的坏死、脂膜炎，在某些病例中还表现为不典型的淋巴样细胞。组织学特征的共性提示了共同的病理生理学基础，可能是由EBV诱导的细胞因子和趋化因子介导的。一小部分病变(15%)表现为硬化和萎缩的斑块，在所研究的少数病例中未发现EBV。斑块样病变与LYG的关系仍不确定。虽然一些LYG病例会自发消退，但大多数需要治疗。
Lymphomatoid granulomatosis (LYG) is a rare angiocentric and angiodestructive Epstein-Barr virus-associated B-cell lymphoproliferative disorder (EBV-BLPD), varying widely from an indolent process to an aggressive large cell lymphoma. The skin is the extrapulmonary organ most commonly involved in LYG. We studied 32 skin lesions from 20 patients with known pulmonary LYG, using immunohistochemistry, in situ hybridization for EBV, and polymerase chain reaction for the presence of antigen receptor gene rearrangements (IgH and TCR) to better define both the clinicopathologic spectrum and pathogenesis of the cutaneous lesions. We describe two distinct patterns of cutaneous involvement. Multiple erythematous dermal papules and/or subcutaneous nodules, with or without ulceration, were present in 17 patients (85%). These lesions demonstrate a marked angiocentric lymphohistiocytic infiltrate, composed predominantly of CD4-positive T-cells, with a high propensity for involving the subcutaneous tissues, and exhibiting angiodestruction, necrosis, and cytologic atypia. EBV-positive B-cells were detected in the nodules from five patients', clonal immunoglobulin heavy chain gene (IgH) rearrangements were detected by polymerase chain reaction in two patients. Multiple indurated, erythematous to white plaques were present in three patients (15%). The plaque lesions were negative for EBV and clonal IgH gene rearrangements in all cases studied. The clinical course of overall disease was variable, ranging from spontaneous regression without treatment (1 of 13; 7%), resolution with chemo/immunomodulatory therapy (8 of 13, 62%), and progression (4 of 13; 31%). The clinical and histopathologic features of cutaneous LYG are extremely diverse. However, the majority (85%) of the cutaneous lesions mirrors to some extent LYG in the lung, although EBV+ cells are less frequently identified. This subset of cases shows the histopathologic triad of angiodestruction with associated necrosis, panniculitis, and in some cases atypical lymphoid cells. The commonality of the histologic features in this group suggests a common pathophysiologic basis, possibly mediated by cytokines and chemokines induced by EBV. A small percentage of the lesions (15%) presented as indurated and atrophic plaques, and EBV was not identified in the small number of cases studied. The relationship of the plaque-like lesions to LYG remains uncertain. Whereas some cases of LYG regress spontaneously, most require therapy.