CREST syndrome
In the CREST syndrome is a special form of scleroderma.
The name of this syndrome derives from the English words from the symptoms listed features for this disease.
The cause of this disease is still unknown. It is estimated that over a unnbekanntes autoantigen in the skin is caused by an infiltration of T lymphocytes. So it is then over several intermediate steps in increased collagen synthesis in the skin.
Frequency: see scleroderma
Symptoms
- Calzinosis (connective tissue calcification)
- Raynaud's phenomenon (paroxysmal less supply the finger with blood)
- Esophageal dysmotility
- Sclerodactyly (thin, pale fingers)
- Telangiectasia (enlargement of the small, superficial skin vessels)
Diagnosis: see scleroderma
The specific diagnosis of CREST syndrome arises from the clinical picture and the possible detection of antibodies against Centromere structures
Complications: see scleroderma
In contrast to the diffuse form of scleroderma can be limited in time Autret remissions.
Therapy
- Treatment of Raynaud's syndrome with calcium channel blockers (nifedipine)
- intensive physical therapy and physiotherapy
- The importance of steroid therapy is controversial because it affects the disease process is generally scarce.