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[Vascular lesions and fibroblast metabolism in inflammatory diseases].

Alterations of the vascular endothelium are frequent in sclerodermis and are manifested by signs of tissue repair. In these patients, the serum seems to contain a cytotoxic component while their platelets are very adhesive to the collagen. During the phase of activity of the disease, sclerodermic fibroblasts show an increased collagen biosynthesis. The vascular lesion could be responsible for the migration of mobile cells of which the mediators (lymphokins, monokins, PDGF, etc...) would activate the fibrocytes.

Autoantibodies↗

Mast cell numbers in diffuse scleroderma.

Numbers of mast cells were quantitated in the lesions of diffuse scleroderma and morphea. Mast cells increased and then decreased in number in the papillary dermis of diffuse scleroderma. No significant change of mast cell numbers was noted in the reticular dermis. Mast cells increased in the papillary dermis with fine collagen bundles (grade 2 skin of scleroderma) and decreased in the papillary dermis with homogeneous collagen bundles (grade 3 skin of scleroderma). The total number of cells increased in the papillary dermis of grade 1 and 2 skin of scleroderma and decreased in the grade 3 skin of scleroderma. In morphea a reduced number of mast cells was noted in grade 3 lesions. It is suggested that mast cells play an important role in fibrotic process of scleroderma skin.

Cell Count↗

[Association of HLA antigens with scleroderma].

22 patients suffering from scleroderma have been investigated with regard to possible association of HLA with scleroderma (PSS and s. circumscripta). Preliminary data revealed increased relative risk values for carriers of the HLA antigens DR1 and DR5 (rR value 6.3 and 2.6 for PSS, resp.). In s. circumscripta, the rR value of individuals positive for B27 was increased up to 4.9. Different subtypes of scleroderma seem to be associated with different HLA antigens.

Cytotoxicity Tests, Immunologic↗

[Connective tissue compartments in scleroderma. Study of the structure and the biomechanical properties].

A combined histological and biomechanical study was performed in patients with localised or systemic scleroderma. The inflammatory reaction was variable in intensity and location, predominating around the superficial vascular plexus, the deep dermo-hypodermal plexus, the paraseptal plexus or in the fasciae. Secondary sclerosis resulted from the lateral fusion of bundles of collagen fibers. Three degrees of intensity of sclerosis were disclosed by our noninvasive technique of tonometry by vertical traction of the skin in vivo. That measurement allowed a follow-up of the evolution of the disease and a control of the therapeutic efficacy.

Connective Tissue↗

Cutaneous fibrinolytic activity in scleroderma.

Fibrinolytic activity is notoriously related to the maintenance of an adequate microcirculatory blood flow and its impairment is related to thrombosis. In this report cutaneous fibrinolytic activity has been evaluated with an autohistographic method in ten subjects with morphea and in five subjects with systemic sclerosis before and after injection of histamine into the skin. Cutaneous fibrinolytic activity (CFA) appeared normal (3/5 cases) or increased (2/5) in uninvolved and involved skin in systemic scleroderma and always increased after histamine injection. In circumscribed scleroderma the CFA appeared reduced in 2/10 cases, normal in 4/10 cases and increased in 4/10 cases. These data show that an endothelial pool of plasminogen activator is available in the skin of scleroderma patients and that fibrinolytic agents can rationally be employed only in certain stages of the disease.

Adult↗