Scleroderma

Scleroderma – Chronic Connective Tissue Disease

Scleroderma is a chronic connective tissue disease which is classified as one of the autoimmune rheumatic diseases wherein the body tends to attack its own tissues. It results in hard, thickened areas of skin and at times with problems with internal organs as well as the blood vessels.

Though the cure is unknown, people still tend to lead a full productive life. The word `scleroderma’, comes from two Greek words `sclero’, which means hard and `derma’ means skin. In this case the hardening of the skin is the most obvious sign of the disease which has been called progressive systemic sclerosis though the term has been discouraged since the scleroderma is not really progressive and varies from person to person.

Scleroderma is not contagious or infectious, cancerous or malignant. Based on the form of the disease, it could affect the skin by few thickened oval patches which may take place in any area of the body or one may find lines of thickened skin on the face, scalp, legs or forearms. Other possibilities of this condition would depend on which internal organ is affected.

For milder form of this condition, treatment would not be essential. There are two types of scleroderma namely localised scleroderma which may affect only the skin and systemic sclerosis which may affect the circulation of the blood as well as internal organs together with the skin.

Types of scleroderma

Milder form is the localised scleroderma and may take place at any age wherein the disease only seems to affect the skin with one or more hard patches while the internal organs are not affected.

 Localised scleroderma is of two kinds namely Morphoea and Linear. In the case of Morphoea, one will find small oval skin patches, which tend to be itchy, may affect skin in any area of the body, patches could be hairless or lighter/darker than the normal skin, and patches may tend to fade after a few years, while in Linear form of scleroderma, one will have thickened skin which appears in lines across the face or scalp leg or arm, lines tend to persist longer than morphoea patches, occasionally affecting underlying bone and muscle, could lead to growth problems in the case of children and may cause deformity.

Consultation/Specialized Test

People with systemic sclerosis tend to have problems with their gut like heartburn and difficulty in swallowing. The skin begins to get puffy and thickens restricting joint movement while toes and fingers may begin to get swollen before the skin tends to get hard and tight.

Besides, the facial skin too gets tight especially around the mouth area. Systemic sclerosis commonly occurs in adults in the age group of 30 and 50 affecting women more than the men with rare instances of about only 10% of children being affected with this ailment.

The process of diagnosis may involve consultation with rheumatologist or dermatologist with blood studies along with other specialized test based on which organ is affected.

 Treatment is carried out to relieve symptoms and to prevent the disease from getting worse, to identify and treat any other complication which may arise like pulmonary hypertension, minimum disability through occupational therapy and physiotherapy.