SCLERODERMA (BASED ON A STUDY OF OVER 150 CASES)

Abstract

A review of over 150 patients with scleroderma on whom a multitude of studies were performed. This is one of the diseases of the mesenchyme of unknown etiology. The many theories are reviewed. It is more common in females, almost 3 to 1. It does not appear to be hereditary. The systemic nature of the disease is emphasized. Laboratory tests are indicative of the tissue or organ involved in the sclerotic process. Tissues derived from the embryonic mesenchyme may be involved anywhere in the body. Lesions are frequently seen in the gastrointestinal tract, lungs, heart, kidneys, blood vessels, as well as the skin. Subjective and objective findings are described. In general, the lesions found in scleroderma are of 3 types. (1) Changes in the collagen in various parts of the body. (2) Changes in the arteries such as acute arteritis, an obliterative arteriolar lesion, and a sclerotic lesion often seen secondary to hypertension but involving a slightly larger vessel of the kidney. (3) The immediate and remote results of these lesions, comprising such changes as fibrosis, parenchymatous degeneration, infarction, etc. The symptoms are a result of these lesions. The course may be fulminating becoming rapidly fatal, or remain in an arrested state throughout an average life span. There may be periods of arrest and exacerbation. In a small series of very severe cases, cortisone appeared to arrest the course of the disease when used continuously in fairly large doses. In several patients it appeared to be a life-saving procedure.