Sclerodermais the prototype of the fibrotic skin diseases. It may manifest either as systemic and localized forms. Progressive systemic sclerosis (PSS) is a generalized connective tissue disorders, the fibrotic process of affecting not only the skin but also the heart, lungs, kidneys, and the gastrointestinal tract. The overall female to male incidence of scleroderma is 3:1. The average age of onset is 40 to 50 years. In women, however, the average age of onset lies in the child bearing age between 30 and 39. Men and older patients with systemic scleroderma tend to have a shorter survival rate and thus a poorer prognosis.
Clinical FeaturesSystemic cleroderma may involve all connective tissue containing organs; however, some represent the main target of the disease. These include the skin, gastrointestinal tract, heart, lungs, and kidneys.
Cutaneous Manifestationsare usually early symptoms. They may either proceeds or accompany Raynaud's disease.They may be divided into an early edemaous phase, sclerotic phase, and late atrophic phase.
Raynaud's phenomenonIt occurs in over 90 percent of patients with scleroderma and may be the initial symptom. It is characterized by a triphasic color reaction involving the distal digits.
Gastrointestinal tractMost systemic scleroderma patients may develop dysphagia, regurgitation, peptic esophagitiss, and fibrotic strictures.
HeartPrimary cardiac involvement is common in systemic scleroderma. However, it is frequently overlooked.
LungsPulmonary involvement is one of the most important clinical features in systemic scleroderma. In addition to X- ray procedures and functional tests, scintigraphy and the bronchoalveolar lavage have offered new insights into the pathophysiologic events.
Kidneys The presence of renal involvement in scleroderma is regarded as one of the features associated with the poorest prognosis. The clinical criteria for renal disease in scleroderma include persistent proterinuria (> 500 mg/ 24 hr), hypertension (> 140/ 90 mm Hg), and azotemia (blood urea nitrogen BUN > 25 mg/ 100 mL).
