Various Manifestations of Rheumatic Disorders: Case 6 Progressive Systemic Sclerosis

For several months, a 70-year-old woman had had dysphagia, mild dyspnea on exertion, and the Raynaud phenomenon. Her skin was waxy and edematous; 2- to 10-mm pinkish spots had appeared on her fingers, palms, and oral mucous membrane over the past 2 weeks. These disappeared completely with pressure. Subcutaneous calcific deposits were present on the extensor surfaces of the forearms. The spots are telangiectases of progressive systemic sclerosis. The Raynaud phenomenon is seen in about 90% of cases, and skin thickening, pigment changes, and internal organ fibrosis are important characteristics. Patients with limited systemic scleroderma frequently have calcinosis cutis, the Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia (CREST syndrome), as this woman did. In such patients (compared with those who have diffuse systemic scleroderma), skin tightening is confined to the hands and face; there is a lower risk of renal involvement, a higher risk of pulmonary hypertension, and an overall better prognosis. In diffuse systemic sclerosis, the course of visceral disease is more rapid, leading to death. Laboratory findings disclose a variety of abnormalities. There may be mild anemia, hypergammaglobulinemia (in 50% of patients), proteinuria, positive tests for antinuclear antibodies, presence of anticentromere antibody (in 50% of those with CREST syndrome and 1% of those with diffuse systemic scleroderma), and Scl-70 antibodies (in 20% of those with CREST syndrome and 33% of those with diffuse disease). Diseases that must be considered in the differential diagnosis include eosinophilic fasciitis (which is not associated with the Raynaud phenomenon, manifests peripheral blood eosinophilia, and responds to prednisone( therapy); porphyria cutanea tarda; chronic graft-versus-host disease; and eosinophilia-myalgia syndrome secondary to tryptophan( ingestion.

(Case and photograph courtesy of Dr Shanon D. Smith.)