Systemic Sclerosis

The face in diffuse Systemic Sclerosis: note the radial furrows surrounding the mouth, pinched nose and scattered telangectasia.


Clinical Background:

This patient had a 20 year history of Raynaud's phenomenon and hand stiffness; in the last few years she noticed the appearance of several telangectasia on the skin and started suffering from dysphagia and heartburn. For one year before coming to our Department she had been treated with nifedipine 20 mg/d and griseofulvin 500 mg/d.

Physical examination revealed sclerodactyly and pitted scars on the finger-tips, widespread telangectasia involving the face, upper trunk and extremities, microstomia with angular creases, radial furrows and mild hypertension.
EGDS showed severe esophagitis, distal esophageal dilation and hiatus hernia. Esophageal manometry showed decreased amplitude and incoordination of peristaltic waves in the distal esophagus and inadequate lower esophageal sphincter competence. In addition 24-hour pH monitoring showed an abnormal acid exposure of the distal esophagus and decreased clearing ability.
Chest radiograph and pulmonary function tests (including carbon monoxide diffusing capacity) were normal. She had no electrocardiographic or echocardiographic evidence of cardiac involvement. Urinalysis and other indicators of renal function were within normal limits.
She was discharged on penicillamine (150 mg/d), nifedipine (20 md/bid) and gastric antacids.

All contents copyright of the "On-line Archives of Rheumatology", 1996