Department of Pediatrics, Ain Shams University, Cairo, Egypt.
Allergy Asthma Immunol Res. 2011 Apr;3(2):138-40. doi: 10.4168/aair.2011.3.2.138. Epub 2011 Feb 9.
The β2 integrins are expressed exclusively on leukocytes and participate in many immune and inflammatory processes. This subfamily comprises four heterodimeric glycoproteins with a common β-subunit, designated β2 (CD18). Spontaneous mutations of the CD18 gene result in leukocyte adhesion deficiency type I (LAD-I). Low level of CD18 expression has also been implicated in the pathogenesis of psoriasis. We here describe a child with recurrent skin infections without pus formation, persistent gingivitis and periodontitis. His blood counts showed persistent leukocytosis (neutrophilia). CD11b expression was defective on neutrophils, while that of CD18 was normal. So, our patient represents a mild variant of LAD-I with possible dysfunctional CD18. Moreover, he developed psoriasis with reduced CD18 expression on CD4(+) T-cells. Psoriasiform dermatitis has been described before in association with LAD-I, however, clinically and histologically confirmed psoriasis in association with LAD-I has been described only in CD18 hypomorphic mice. Therefore, our patient represents the first clinically and histopathologically documented association between LAD-I and psoriasis in humans. It lends support to the role of β2 integrins in the etiopathogenesis of psoriasis.
β2 整合素仅表达于白细胞上,并参与多种免疫和炎症过程。该亚家族由四个具有共同的β亚单位(称为β2(CD18))的异二聚体糖蛋白组成。CD18 基因突变会导致白细胞黏附缺陷症 I 型(LAD-I)。CD18 表达水平低也与银屑病的发病机制有关。我们在此描述了一例反复发生无脓液形成的皮肤感染、持续性牙龈炎和牙周炎的患儿。他的血细胞计数显示持续的白细胞增多(中性粒细胞增多)。中性粒细胞上的 CD11b 表达有缺陷,而 CD18 的表达正常。因此,我们的患者代表了 LAD-I 的轻度变异型,可能存在功能失调的 CD18。此外,他还出现了银屑病,CD4+T 细胞上的 CD18 表达减少。以前曾描述过 LAD-I 与银屑病样皮炎之间的相关性,但仅在 CD18 低功能的小鼠中描述过与 LAD-I 相关的临床和组织学确诊的银屑病。因此,我们的患者代表了人类 LAD-I 和银屑病之间首次在临床和组织病理学上得到证实的关联。它支持了β2 整合素在银屑病发病机制中的作用。