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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.
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