Rivera Francisco, Anaya Sara, Pérez-Alvarez Javier, de la Nieta Maria D Sánchez, Vozmediano María C, Blanco Julia
Sección de Nefrología, Hospital General de Ciudad Real, c/Tomelloso s/n, 13005 Ciudad Real, Spain.
J Med Case Rep. 2010 Feb 11;4:50. doi: 10.1186/1752-1947-4-50.
Henoch-Schönlein purpura is a systemic disease with frequent renal involvement, characterized by IgA mesangial deposits. Streptococcal infection can induce an abnormal IgA immune response like Henoch-Schönlein purpura, quite similar to typical acute post-infectious glomerulonephritis. Indeed, hypocomplementemia that is typical of acute glomerulonephritis has also been described in Henoch-Schönlein purpura.
We describe a 14-year-old Caucasian Spanish girl who developed urinary abnormalities and cutaneous purpura after streptococcal infection. Renal biopsy showed typical findings from Henoch-Schönlein purpura nephritis. In addition, she had low serum levels of complement (C4 fraction) that persisted during follow-up, in spite of her clinical evolution. She responded to treatment with enalapril and steroids.
The case described has, at least, three points of interest in Henoch-Schönlein purpura: 1) Initial presentation was preceded by streptococcal infection; 2) There was a persistence of low serum levels of complement; and 3) There was response to steroids and angiotensin-converting enzyme inhibitor in the presence of nephrotic syndrome. There are not many cases described in the literature with these characteristics. We conclude that Henoch-Schönlein purpura could appear after streptococcal infection in patients with abnormal complement levels, and that steroids and angiotensin-converting enzyme inhibitor could be successful treatment for the disease.
过敏性紫癜是一种常累及肾脏的全身性疾病，其特征为IgA系膜沉积。链球菌感染可诱发类似过敏性紫癜的异常IgA免疫反应，与典型的急性感染后肾小球肾炎颇为相似。事实上，急性肾小球肾炎典型的低补体血症在过敏性紫癜中也有描述。
我们描述了一名14岁的西班牙裔白种女孩，她在链球菌感染后出现了泌尿系统异常和皮肤紫癜。肾活检显示为过敏性紫癜性肾炎的典型表现。此外，尽管她的病情有好转，但随访期间血清补体（C4组分）水平一直较低。她对依那普利和类固醇治疗有反应。
所描述的该病例在过敏性紫癜方面至少有三点值得关注：1）最初表现之前有链球菌感染；2）血清补体水平持续较低；3）在肾病综合征存在的情况下对类固醇和血管紧张素转换酶抑制剂有反应。文献中报道的具有这些特征的病例并不多。我们得出结论，在补体水平异常的患者中，链球菌感染后可能出现过敏性紫癜，并且类固醇和血管紧张素转换酶抑制剂可能是该疾病的成功治疗方法。
