S8C33P19-CT Angiopathies(hyaline)

S8C33P19-1: In the category of connective tissue diseases, the designation, collagen-vascular disease, implies that vascular disease plays a role in pathogenesis. In cutaneous lesions of scleroderma, muscular vessels in the deeper portions of the dermis are often associated with perivascular infiltrates of lymphocytes and histiocytes - often with a component of plasma cells.  The infiltrates may involve the walls of muscular veins and also may be found in the sub-endothelial space of such vessels. Generalized scleroderma is a multisystem disease. In the viscera, muscular vessels tend to show mucinous hyperplasia of the intima. They may also show fibrosis and hyalinization of the media. Pulmonary lesions, as seen in this case, are characterized by activation of connective tissue cells, and by the deposition of intercellular matrix. As a consequence, pulmonary function is compromised. Early on, such lesions may show a mucinous hyperplasia of the alveolar walls. Eventually, the alveolar walls become fibrotic as in the above field. Two vessels, to the left in this field, show narrowed lumens and fibrosis of the intima and the media.

S8C33P19-2: Late cutaneous lesions of scleroderma, or morphea, show an expansion of the fibrous domain at the expense of a portion of the domain of the adipose tissue. This elastic stain provides a sharp definition between the altered, but pre-existing, reticular dermis (the domain in which elastic fibers are preserved) and the area of substitutive fibrosis (the area in the bottom 1/2 of the field in which elastic fibers are not represented). The dense fibrous tissue in the area of substitutive fibrosis is relatively avascular. Some of the areas, that are devoid of elastica, may represent foci, which, at an earlier stage in the evolution of the process, would have been characterized by inflammatory infiltrates and inflammatory elastolysis.

 

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