If CREST syndrome is suspected, it is important to look for its cardinal features. Cutaneous calcinosis usually presents over the bony prominences of knees, elbows, spine, and iliac crests, and may be painful. Patients may complain of Raynaud’s phenomenon with triphasic color changes, ie, pallor, cyanosis, and rubor, occurring months to years before sclerosis. Ulcerations at fingertips from Raynaud’s may be evident as pitted scars on physical exam. There is also a nonpitting edema of hands and feet that later progresses into sclerodactyly with tapering of fingers (our patient actually had clubbing). Patients may complain of stiffness of the hands and feet as the sclerosis progresses.
As a result of the edema and fibrosis of the face, patients may lose facial lines and comment that they look younger. Often, they will indicate that they have noticed the appearance of small blood vessels on their face, mouth, or hands. Patients may also complain of gastrointestinal problems such as esophageal reflux, diarrhea, or dysphagia.
TABLE 1
Characteristics of systemic scleroderma4-6
| Diffuse systemic scleroderma | Limited systemic scleroderma (Includes CREST syndrome) | |
|---|---|---|
| Constitutional | Fatigue and weight loss or gain | None |
| Vascular | Mild to moderate Raynaud’s phenomenon | Moderate to severe Raynaud’s phenomenon |
| Cutaneous | Sclerosis to trunk, arms, and face; rapid progression | Sclerosis to hands or toes and face; slow progression; calcinosis is prominent |
| Musculoskeletal | Arthralgias and deformities, muscle weakness, and tendon friction rubs | Arthralgias |
| Gastrointestinal | GERD, esophageal dysmotility, and malabsorption are common; all may be severe | Mild to moderate GERD and esophageal dysmotility are common; malabsorption is less common |
| Renal | Severe hypertension, and renal infarcts in renal crisis are common | Rare |
| Pulmonary | Pulmonary hypertension and interstitial lung disease are common | Uncommon |
| Cardiac | Cardiomyopathy, heart failure, and arrhythmias are common | Uncommon |
| GERD, gastroesophageal reflux disease. | ||