title: Factor XIIIa-positive dendrocyte rarefaction in Ehlers-Danlos syndrome, classic type.
abstract: The Ehlers-Danlos syndrome (EDS) represents a heterogeneous group of connective tissue disorders recognized by distinct clinical, microscopic, and biochemical aspects. In particular, some histologic and ultrastructural clues have been reported in the literature. We present a novel immunohistochemical aid to the diagnosis of the most frequent type of EDS. Factor XIIIa-positive dendrocytes are almost absent in the reticular dermis and markedly reduced in number and size in the adventitial dermis. By contrast, the densities of vimentin-positive cells and CD34-positive cells were unremarkable. The biologic significance of this finding is unknown. However, at least a subset of dermal dendrocytes interacts with fibroblasts. This mechanism could be affected in the classic form of EDS.
authors.affiliation: Department of Dermatopathology, University Medical Center Sart Tilman, Liège, Belgium.
authors.forename: T
authors.initials: T
authors.lastname: Hermanns-Lê
authors.forename: G E
authors.initials: GE
authors.lastname: Piérard
mesh_terms: D000293:Adolescent; D000328:Adult; D018952:Antigens, CD34 / Q000032:analysis; D015415:Biomarkers / Q000032:analysis; D002648:Child; D003713:Dendritic Cells / Q000737:chemistry* / Q000648:ultrastructure; D020405:Dermis / Q000473:pathology; D004535:Ehlers-Danlos Syndrome / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D026122:Factor XIIIa / Q000032:analysis*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D014746:Vimentin / Q000032:analysis
chemical_list: D018952:Antigens, CD34; D015415:Biomarkers; D014746:Vimentin; D026122:Factor XIIIa