Limited Scleroderma : CREST Syndrome

Comments:
Cutaneous manifestations in CREST syndrome are usually distal to the metacarpophalangeal joints. Note the sausage shaped fingers with tightly stretched skin and telangiectasia. There is beginning of flexion contracture in the middle finger.

Many patients with limited cutaneous sclerosis fail to meet all the criteria for CREST syndrome. On the other hand, CREST manifestations may be sometimes seen in patients with diffuse disease. Hence, the application of the designation 'CREST Syndrome' does not have the same clinical connotation that it once did.

Long-term Complications of CREST Syndrome: Patients are at risk of developing pulmonary hypertension, Sjogren's syndrome, biliary cirrhosis, discoid lupus erythematosus, Hashimoto's thyroiditis, and Graves' disease.