[Electron microscopy study of pathological collagen fibril formation in systemic scleroderma].
Electron microscopy of the skin biopsies from patients with systemic sclerodermia revealed the pathologic alterations of cellular and intracellular structures. The disease is characterized by focal lysis of nuclear, organoid and cytoplasmic membranes of the fibroblastic cells with the most essential alteration of cell plasmalemma. The cellular fragments (parts of cytoplasma, cisterns of the rough endoplasmic reticulum, mitochondria, nuclear debris) are found between the extracellular bundles of collagen fibrils. The pathologic forms of collagen aggregation (globular forms, non-homogeneous fibrils of the quaternary structure and transversally-bound microfibrillar aggregates) appear along with the fibrillar quaternary structures. Abnormal forms of fibrillar collagen are reluctant for deaggregation and lysis and facilitate the progression of fibrosis.
D001327:Autoimmune Diseases / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D001706:Biopsy; D003094:Collagen / Q000276:immunology / Q000378:metabolism*; D003238:Connective Tissue / Q000276:immunology / Q000378:metabolism / Q000648:ultrastructure*; D006651:Histocytochemistry; D006801:Humans; D007111:Immunity, Cellular; D008854:Microscopy, Electron; D012595:Scleroderma, Systemic / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D012867:Skin / Q000276:immunology / Q000378:metabolism / Q000648:ultrastructure
D003094:Collagen
Elektronno-mikroskopicheskoe izuchenie patologicheskogo fibrilloobrazovaniia kollagena pri sistemnoĭ sklerodermii.