title: Decreased expression of decay-accelerating factor on endothelial cells of immune complex-mediated vasculitic skin lesions.
abstract: Endothelial cells may be damaged directly by the membrane attack complex of complement in immune complex vasculitis of the skin. However, for endothelial cell membrane injury to occur, normal regulatory mechanisms must fail. One of the main complement regulatory proteins of endothelial cells is decay-accelerating factor, a surface protein which interferes with either the classical or alternative pathway C3 and C5 convertases. We have investigated the expression of decay-accelerating factor in 4 patients with histologically proven cutaneous immune complex vasculitis, using an immuno-electronmicroscopic technique. We demonstrated that endothelial cells of upper dermal vessels in vasculitic lesions were almost completely devoid of decay-accelerating factor. By contrast, the expression of this protein on endothelial cells in uninvolved skin of the patients was the same as in skin of healthy volunteers. As yet, the mechanism responsible for depletion of decay-accelerating factor is not clear. Absence of decay-accelerating factor may follow enzymatic release from the phosphatidylinositol anchor, proteolytic stripping from the cell membrane or a down-regulation of decay-accelerating factor synthesis. Regardless of mechanism, endothelial cell injury or death could serve a phlogistic function to facilitate complement-mediated destruction of endothelial cells for removal and repair.
authors.affiliation: Department of Dermatology, University Hospital, Leiden, The Netherlands.
authors.forename: B W
authors.initials: BW
authors.lastname: Boom
authors.forename: A M
authors.initials: AM
authors.lastname: Mommaas
authors.forename: M R
authors.initials: MR
authors.lastname: Daha
authors.forename: B J
authors.initials: BJ
authors.lastname: Vermeer
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000936:Antigen-Antibody Complex / Q000502:physiology*; D018958:CD55 Antigens; D003169:Complement Inactivator Proteins / Q000378:metabolism*; D004727:Endothelium / Q000166:cytology* / Q000378:metabolism / Q000648:ultrastructure; D005260:Female; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000378:metabolism*; D016253:Microscopy, Immunoelectron; D012867:Skin / Q000166:cytology / Q000378:metabolism / Q000648:ultrastructure; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology
chemical_list: D000936:Antigen-Antibody Complex; D018958:CD55 Antigens; D003169:Complement Inactivator Proteins; D008565:Membrane Proteins