Pfaltz Katrin, Mertz Kirsten, Rose Christian, Scheidegger Paul, Pfaltz Madeleine, Kempf Werner
Kempf & Pfaltz Histological Diagnostics, CH-8042 Zürich, Switzerland.
J Cutan Pathol. 2010 Jun;37(6):654-8. doi: 10.1111/j.1600-0560.2009.01450.x. Epub 2009 Oct 15.
Direct immunofluorescence (DIF) testing is an important procedure in the diagnosis of autoimmune bullous dermatoses. We investigated the expression of C3d in formalin-fixed, paraffin-embedded tissue of autoimmune bullous dermatoses.
The immunohistochemical expression of C3d in bullous pemphigoid (BP) (n = 32), pemphigoid gestationis (PG) (n = 3), pemphigus (n = 14), dermatitis herpetiformis Duhring (DHD) (n = 10), linear immunoglobulin A (IgA) dermatosis (n = 4), mixed forms of BP and linear IgA dermatosis (n = 2), and 44 controls was analyzed on formalin-fixed tissue.
Thirty-one of 32 cases (97%) of BP and 3 out of 3 cases (100%) of PG showed a linear positivity of C3d along the basement membrane. Only 3 out of 14 (21%) cases of pemphigus showed an intraepidermal intercellular expression of C3d. The two mixed forms of linear IgA dermatosis and BP showed a linear positivity of C3d along the basement membrane. All cases of DHD, linear IgA dermatosis and all of the controls were negative for C3d.
C3d immunohistochemistry is a valuable tool in the diagnosis of BP and PG of the skin with a sensitivity of at least 97%. Mixed forms of linear IgA dermatosis, and BP, DHD and linear IgA dermatosis can only be identified by DIF. A positive result may prompt serologic confirmation of BP without further need for DIF.
直接免疫荧光(DIF)检测是自身免疫性大疱性皮肤病诊断中的一项重要程序。我们研究了C3d在自身免疫性大疱性皮肤病福尔马林固定、石蜡包埋组织中的表达情况。
分析了大疱性类天疱疮(BP)(n = 32)、妊娠类天疱疮(PG)(n = 3)、天疱疮(n = 14)、杜林疱疹样皮炎(DHD)(n = 10)、线状免疫球蛋白A(IgA)皮病(n = 4)、BP与线状IgA皮病的混合形式(n = 2)以及44例对照的福尔马林固定组织中C3d免疫组化表达情况。
32例BP中有31例(97%)和3例PG中有3例(100%)显示C3d沿基底膜呈线状阳性。14例天疱疮中仅3例(21%)显示C3d在表皮内细胞间表达。线状IgA皮病和BP的两种混合形式显示C3d沿基底膜呈线状阳性。所有DHD、线状IgA皮病病例及所有对照的C3d均为阴性。
C3d免疫组化是诊断皮肤BP和PG的一种有价值的工具,敏感性至少为97%。线状IgA皮病与BP的混合形式、DHD和线状IgA皮病只能通过DIF来鉴别。阳性结果可促使对BP进行血清学确认,无需进一步进行DIF检测。