Patients with limited scleroderma will have had Raynaud's for years, occasionally decades and there will be skin involvement to the hands, face, feet and forearms.

In the early 1900’s several physicians noted a group of features often seen when they examined patients with scleroderma (systemic sclerosis). They coined the term CREST syndrome. This is now known as limited cutaneous systemic sclerosis.

The word CREST stands for 5 symptoms which patients may experience:

C - Calcinosis which is an accumulation of calcium below the outer layer of the skin.

R - Raynaud’s phenomenon - a condition in which the blood supply to the extremities, usually the fingers and toes, is temporarily interrupted.

E - Esophageal (gullet) involvement, causing difficulty in swallowing or indigestion.

S - Sclerodactyly is when the skin of the digits becomes thin, shiny and leathery looking. Fingers and toes may become flexed and stiff.

T - Telangiectasia is the appearance of small blood vessels near the surface of the skin. These can be seen on the fingers, palms, lips, face, tongue and chest wall.

Patients with limited disease need checking every 6-12 months, depending on the stability of the disease. Pulmonary hypertension is recognised as a lethal complication of all forms of systemic sclerosis but especially in the limited form of the condition.

To download further information on Limited Cutaneous Systemic Sclerosis (lcSSc) click here
For information on calcinosis click here.