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