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 pinkishspots had appeared on her fingers, palms, and oral mucousmembrane over the past 2 weeks. These disappearedcompletely with pressure. Subcutaneous calcific depositswere present on the extensor surfaces of the forearms.The spots are telangiectases of progressive systemicsclerosis. The Raynaud phenomenon is seen in about 90%of cases, and skin thickening, pigment changes, and internalorgan fibrosis are important characteristics.Patients with limited systemic scleroderma frequentlyhave 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 ofpulmonary hypertension, and an overall better prognosis.In diffuse systemic sclerosis, the course of visceral diseaseis 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 antinuclearantibodies, presence of anticentromere antibody(in 50% of those with CREST syndrome and 1% of thosewith diffuse systemic scleroderma), and Scl-70 antibodies(in 20% of those with CREST syndrome and 33% of thosewith diffuse disease).Diseases that must be considered in the differentialdiagnosis include eosinophilic fasciitis (which is not associatedwith the Raynaud phenomenon, manifests peripheralblood eosinophilia, and responds to prednisone therapy);porphyria cutanea tarda; chronic graft-versus-host disease;and eosinophilia-myalgia syndrome secondary totryptophan ingestion.

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