Clinical manifestation

Skin: areas of hyperpigmentation alternating with hypopigmentation; overall appearance of tanned skin persists long after sun exposure; telangiectasias on face, neck, and periungual areas; skin of the hands sometimes edematous or indurated early, later sclerotic stage where skin is tight and shiny, with a loss of hair, decreased sweating, and loss of ability to make a skin fold; starts distally on the fingers; any area of the body ultimately may be involved; calcinosis on the fingers and extremities; reduced oral aperture (microstomia) from perioral involvement

Ears, nose and throat: xerostomia and xerophthalmia; vascular changes - Ray-naud phenomenon triggered by cold, smoking, or emotional stress; infarction and dry gangrene sometimes results from severe vasospasm

Musculoskeletal system: arthralgias and morning stiffness sometimes mimicking other systemic autoimmune diseases; hand and joint function may decline from skin tightening; acroosteolysis (i.e., resorption or dissolution of the distal end of the phalanx) sometimes occurs; flexion contractures

Neurologic system: trigeminal neuralgia; carpal tunnel syndrome Respiratory system: dry rales, indicating fibrosis

Esophageal sphincter incompetence Gastrointestinal system: reflux; esophagitis Barrett metaplasia; candidiasis; watermelon stomach or gastric vascular antral ectasia; primary biliary cirrhosis; malab-

sorption; atrophy of smooth muscle and fibrotic changes leading to decreased peristalsis throughout the gastrointestinal tract Renal system: renal failure; cardiac involvement: indicates poor prognosis; pericardial effusions with cor pulmonale; conduction abnormalities; infiltrative cardiomyopathy

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