Irish Raynaud's & Scleroderma Society

Types of Scleroderma

The Two Types of Scleroderma:

1. Systemic Scleroderma

Scleroderma that spreads throughout the body is called Systemic Sclerosis, and it is referred to as either Diffuse or Limited, depending on which parts of the body are involved. Lung, kidney, gastro-intestinal tract, and heart involvement are most serious complications, and patients should be checked regularly by specialists to ensure that they are getting the right medication.

DIFFUSE Scleroderma can be found at many different sites on the body, where scarred tissue and compromised circulation damage skin, joints, muscles, organs, intestines and other parts in no specific order. Although it cannot be cured, the disease can be controlled effectively by medications specific to the affected area. Organ problems may precede significant skin involvement, and it is important to get specialist care as early as possible.

LIMITED Scleroderma was in the past called CREST, because the pattern of involvement is usually limited to five symptoms that spell out these letters.

Calinosis is an accumulation of calcium in little lumps under the skin that protrude painfully, often on the fingers.

Raynaud’s causes ulcers on the fingers and toes when impaired circulation damages the tissues and prevents healing.

Esophageal (US spelling) problems make eating and swallowing difficult, and digestion is impaired by reflux from the stomach.

Sclerodactyly results in leathery fingers and toes.

Telangiectasia gives a spidery red look to areas of skin where tiny capillaries are dilated.

All the symptoms of Limited disease can be reduced by medical intervention, and regular checkups will ensure up-to-date treatment.

2. Localised Scleroderma

Localised Scleroderma is most often seen in children, who may be referred by their GP to a dermatologist for diagnosis of patches of stiff, thickening skin. It can be a crippling disease but drug therapy and physiotherapy have proved successful in delaying its progress. It can also go into remission spontaneously, although its effects are irreversible. It takes two forms, occasionally combined:

MORPHEA develops as one or many slowly enlarging patches of plaques. These are usually oval in shape but vary in size and colour, and may enlarge or shrink, sometimes disappearing spontaneously.

LINEAR Scleroderma can affect the growth of a limb, and afflicted children should be under specialist care. It starts as a band of thickening skin, usually limited to one area of an arm or leg. Unlike morphea, linear Scleroderma usually involves layers of tissue below the skin, affecting the muscles and bones, the mobility of the underlying joints, and the growth rate of the limb. Occassionally linear morphea is found on the head, forming a slash mark across the forehead and scalp. In these cases it is known as “en coup de sabre”.