a. What does the picture show?
c. How can you confirm the diagnosis?
The two main forms of systemic sclerosis are diffuse cutaneous and limited
cutaneous disease. Both forms are more common in women. However, both have
the same spectrum of extra-cutaneous diseases.
Diffuse cutaneous disease. The patients demonstrate Raynaud's phenomenon,
swelling of the hands and feet, hidebound skin changes usually affecting
the trunk. Anti-topoisomerase-1 antibodies (anti-Sci-70) are found in 30%
of patients with diffuse disease.
Limited disease. The skin involvement is limited to the hands, feet,
face, and forearms. The characteristic CREST syndrome (calcinosis, Raynaud's,
oesophageal involvement, and telangiectasia), although seen in both diffuse
and limited disease, occurs most commonly in association with limited cutaneous
systemic sclerosis. Anti-centromere antibodies (ACA) are identified in
80% of patients with limited disease.