title: Morphoea lesions are associated with aberrant expression of membrane cofactor protein and decay accelerating factor in vascular endothelium.
abstract: One of the main features of systemic and localized forms of scleroderma is vascular damage, the mechanism of which is not yet understood. Recently, we have shown undetectable or decreased expression of complement regulatory molecules, membrane cofactor protein (MCP) and decay-accelerating factor (DAF), in cutaneous endothelium of patients with systemic sclerosis (SSc). In some patients, CD59 expression in endothelium was also altered. As these molecules protect endothelial cells from damage by autologous complement, their decreased expression could be part of the mechanism of vascular damage in SSc. In the present study, we investigated the expression of MCP, DAF and CD59 in the endothelium of lesional and non-lesional skin of patients with localized morphoea. Normal skin and lesional skin from patients with systemic lupus erythematosus, and three inflammatory diseases, were included as relevant controls. The results showed that the extent of expression of the three molecules in non-lesional skin of patients with morphoea, on all the skin cells and structures, was identical to that of normal skin. In lesional skin, however, the expression of MCP and DAF in endothelium was either undetectable or only present to a very slight degree. CD59 expression in endothelium in lesional skin was normal. No such aberrant expression was observed in the lesions of any of the control diseases. These results indicate a decreased ability of endothelial cells in lesional areas to protect themselves from autologous complement, and this could contribute to vascular damage in morphoea lesions.
authors.affiliation: Department of Dermatology, University of Amsterdam, Academisch Medisch Centrum, The Netherlands.
authors.forename: G T
authors.initials: GT
authors.lastname: Venneker
authors.forename: P K
authors.initials: PK
authors.lastname: Das
authors.forename: B
authors.initials: B
authors.lastname: Naafs
authors.forename: A J
authors.initials: AJ
authors.lastname: Tigges
authors.forename: J D
authors.initials: JD
authors.lastname: Bos
authors.forename: S S
authors.initials: SS
authors.lastname: Asghar
mesh_terms: D015703:Antigens, CD / Q000032:analysis*; D018958:CD55 Antigens; D018957:CD59 Antigens; D004730:Endothelium, Vascular / Q000276:immunology*; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000276:immunology; D051916:Membrane Cofactor Protein; D008562:Membrane Glycoproteins / Q000032:analysis*; D012594:Scleroderma, Localized / Q000276:immunology*; D012867:Skin / Q000276:immunology*
chemical_list: D015703:Antigens, CD; C497857:CD46 protein, human; D018958:CD55 Antigens; D018957:CD59 Antigens; D051916:Membrane Cofactor Protein; D008562:Membrane Glycoproteins