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Systemic Sclerosis: As the disease progresses, there is skin atrophy and sclerosis of underlying tissues. The skin is bound down and stretched over the subcutaneous tissues. The patients may acquire rigid, expressionless, mask-like faces with beaked nose, thin lips, perioral furrowing, and an inability to fully open mouth.

The diffuse form of systemic sclerosis manifests with widespread cutaneous lesions as well as involvement of the internal viscera such as kidneys, lungs, heart, and the gastrointestinal tract. The prognosis is poor with a 5-yr survival rate ranging from 35% to 75%. It is associated with anti-DNA topoisomerase (Scl-70) and RNA polymerase III antibodies.

Image 33 of 59