CREST Syndrome

Question 1

Classifications of cutaneous systemic sclerosis

Answer 1

Limited cutaneous systemic sclerosis
- Diffuse cutaneous systemic sclerosis
- 2/3rds of patients
- Skin involvement is widespread and includes proximal limbs. Significant internal organ involvement
- Limited cutaneous systemic sclerosis
- 1/3rd of patients
- Limited to digits, distal limbs and face
- Less organ involvement except pulmonary artery hypertension can occur later in disease course

Question 2

Physical Examination of cutaneous systemic sclerosis

Answer 2

• Puffy swollen fingers +/- nonpitting oedema to hands.
• Skin thickening
• Perioral skin tightening
• Abnormal nailfold capillaroscopy with scleroderma pattern (dilated capillary loops, capillary dropout, microhaemorrhages, architectural derangement)
• Calcinosis cutis (hands/elbows/knees)
• Mucocutaneous telangiectasias
• Cutaneous hyperpigmentation
• Tendon friction rubs felt as coarse crepitus over affected joints.