Autoantibodies Complement Mast Cells In Bullous Phemphigoid, Skin Disorder Of The Elderly

Bullous pemphigoid (BP) is a skin disorder of the elderly that is characterized by the presence of fluid-filled blisters on the skin. Individuals with BP have large amounts of antibodies that recognize two of the individual's own proteins (BP180 and BP230), but exactly how these antibodies cause disease has not be determined. Now, in a study using a mouse model of BP, which appears online on October 5 in advance of publication in the November print issue of the Journal of Clinical Investigation, researchers from the University of North Carolina, have shown that these antibodies activate an immune system pathway known as the classical complement pathway.



Transfer of antibodies specific for mouse BP180 to other mice induces the formation of blisters similar to those seen in individuals with BP. However, Zhi Liu and colleagues found that these antibodies do not cause disease in mice lacking C4 (a crucial component of the classical complement pathway). In the absence of activation of the classical complement pathway, immune cells known as mast cells were not activated and neutrophils (another immune cell type) were not recruited to the skin. This study identifies a mechanism by which self-reactive antibodies can cause blister formation in mice, and might lead to new treatments for individuals with BP and other autoimmune blistering diseases, such as herpes gestationis -- which is a nonviral disease of pregnancy.



TITLE: Role of different pathways of the complement cascade in experimental bullous pemphigoid



AUTHOR CONTACT:



Zhi Liu

University of North Carolina, Chapel Hill, North Carolina, USA.



View the PDF of this article at: https://the-jci/article.php?id=17891





JCI table of contents: Oct. 5, 2006



Contact: Karen Honey


Journal of Clinical Investigation