Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Kanagawa, Japan.
J Am Acad Dermatol. 2010 Dec;63(6):1026-9. doi: 10.1016/j.jaad.2009.11.690. Epub 2010 Oct 8.
Henoch-Schönlein purpura (HSP) is a multisystem disease believed to be a consequence of the entrapment of circulating IgA-containing immune complexes in blood vessel walls throughout the skin, kidneys, and gastrointestinal tract. The skin manifestations are characterized by nonthrombocytopenic palpable purpura over the lower extremities.
We assessed adult patients with HSP who had nonthrombocytopenic palpable purpura on the extensor surfaces of their lower limbs, and had no associated connective tissue disease. Patient medical records, including clinical presentation, laboratory data, and direct immunofluorescence (DIF) reports, were reviewed retrospectively.
We reviewed the records of 25 adult patients with HSP who presented at our department, between 2006 and 2008, with an initial cutaneous manifestation of palpable purpura on their lower extremities. Adult HSP was defined in all cases as documented leukocytoclastic vasculitis according to a skin biopsy specimen, with histopathologic evidence of IgA deposition by DIF. Statistical analyses were performed using a χ(2) test to compare prevalence among each clinical manifestation.
There was a significant correlation between IgM deposition by DIF and renal involvement (χ(2) = 5.23, P = .022). IgM deposition and complement 3 deposition by DIF showed a close relationship (χ(2) = 5.11, P = .024). There was a significant positive correlation between serum IgA and C-reactive protein levels (Spearman's rank correlation coefficient = 0.35, P = .044).
These findings should be validated in larger studies. Renal biopsies were not done to confirm the presence of nephritis.
This study suggests that IgM deposition in palpable purpura based on DIF provides an indicator of nephritis in adult patients with HSP. We believe that IgM deposition could be related to the pathogenic factors that trigger the development of renal involvement.
过敏性紫癜(HSP)是一种多系统疾病,被认为是循环 IgA 含免疫复合物在皮肤、肾脏和胃肠道血管壁中嵌顿的结果。皮肤表现为下肢伸侧非血小板减少性可触及紫癜。
我们评估了成人 HSP 患者,其下肢伸侧有非血小板减少性可触及紫癜,且无相关结缔组织病。回顾性分析患者病历,包括临床表现、实验室数据和直接免疫荧光(DIF)报告。
我们回顾了 2006 年至 2008 年期间在我们科就诊的 25 例 HSP 成人患者的记录,这些患者的初始皮肤表现为下肢可触及紫癜。所有病例均根据皮肤活检确诊为白细胞碎裂性血管炎,DIF 有 IgA 沉积的组织病理学证据,诊断为成人 HSP。采用 χ(2)检验比较各临床表现的患病率。
DIF 中 IgM 沉积与肾脏受累有显著相关性(χ(2) = 5.23,P =.022)。DIF 中 IgM 沉积与补体 3 沉积密切相关(χ(2) = 5.11,P =.024)。血清 IgA 与 C 反应蛋白水平呈显著正相关(Spearman 秩相关系数 = 0.35,P =.044)。
这些发现应在更大的研究中得到验证。未行肾活检以确认肾炎的存在。
本研究提示 DIF 检测到的可触及紫癜中的 IgM 沉积可作为 HSP 成人患者肾炎的指标。我们认为 IgM 沉积可能与触发肾脏受累的致病因素有关。