de Jager Michelle, Blokx Willeke, Warris Adilia, Bergers Mieke, Link Monique, Weemaes Corry, Seyger Marieke
Department of Dermatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
J Cutan Pathol. 2008 May;35(5):467-72. doi: 10.1111/j.1600-0560.2007.00854.x. Epub 2008 Jan 14.
Cutaneous granulomas can occur in patients with a primary immunodeficiency disorder. In some cases, an infectious cause cannot be revealed. The pathogenesis of these granulomas still remains to be elucidated. The aim of this study was to study differences or overlap between these rare granulomas and sarcoidosis-related granulomas.
Markers for T-cell subsets (CD3, CD4, CD8 and CD45RO), Langerhans' cells (CD1a), macrophages (CD68), B cells (CD20) and NK cells (CD56) were stained immunohistochemically. The amount of CD4+ and CD8+ cells in the granulomas was counted. Results were compared with the CD4+/CD8+ ratio in peripheral blood.
In the granulomas of two of three patients with a primary immunodeficiency disorder, the cytotoxic T cells (CD8+) outnumbered the T-helper cells (CD4+) with a counted CD4+/CD8+ ratio <<1. In contrast, the granulomas in the cutaneous sarcoidosis patients showed a predominance of CD4+ cells, with CD4+/CD8+ ratios >2.
A lower CD4+/CD8+ ratio was found in the cutaneous granulomas of patients with a primary immunodeficiency disorder (unclassified combined immunodeficiency, autoimmune lymphoproliferative syndrome and ataxia teleangiectasia) as compared with the patients with cutaneous sarcoidosis. The possible implications of these findings are discussed in this paper.
原发性免疫缺陷病患者可出现皮肤肉芽肿。在某些情况下，无法揭示感染原因。这些肉芽肿的发病机制仍有待阐明。本研究的目的是研究这些罕见肉芽肿与结节病相关肉芽肿之间的差异或重叠。
采用免疫组织化学方法对T细胞亚群（CD3、CD4、CD8和CD45RO）、朗格汉斯细胞（CD1a）、巨噬细胞（CD68）、B细胞（CD20）和自然杀伤细胞（CD56）的标志物进行染色。对肉芽肿中CD4+和CD8+细胞的数量进行计数。将结果与外周血中的CD4+/CD8+比值进行比较。
在3例原发性免疫缺陷病患者中的2例患者的肉芽肿中，细胞毒性T细胞（CD8+）数量超过辅助性T细胞（CD4+），计数的CD4+/CD8+比值远小于1。相比之下，皮肤结节病患者的肉芽肿中CD4+细胞占优势，CD4+/CD8+比值>2。
与皮肤结节病患者相比，原发性免疫缺陷病（未分类的联合免疫缺陷、自身免疫性淋巴增殖综合征和共济失调毛细血管扩张症）患者的皮肤肉芽肿中CD4+/CD8+比值较低。本文讨论了这些发现的可能意义。
