Scleroderma and its oral implications

Scleroderma originates from the Greek words sclero meaning hard tissue and derma, which means skin. It is an umbrella term for different autoimmune conditions caused by excess collagen and scarring in the tissues.

The cause of scleroderma is still uncertain; however, an autoimmune response is evident where fibroblasts produce too much collagen. Triggers for this abnormal immune response have been linked with previous viral infections, such as measles, previous radiotherapy treatment, family history of scleroderma and exposure to certain chemicals or drugs.¹

Scleroderma can be divided in to two main forms: localized and systemic. These can then be further subdivided according to severity and which parts of the body are affected (Figure 1). In localized scleroderma, the collagen deposition is restricted to the dermis and subcutaneous tissues.¹ Fat, fascia, bone and muscle may be affected in this form, but internal organs are usually spared. Approximately 1300 people per year are diagnosed with the condition in the UK, with 70% of cases affecting children between the ages of 5 and 10 years. In adults, it is usually seen in the fifth decade, and affects twice as many woman as men.² In the localized form, thickened white plaques are seen on the skin known as plaque morphea. Long-standing plaques present as brown patches on the skin. The area is often smooth, shiny, hairless and tight.³ This condition is rarely life threatening, but can greatly reduce quality of life as the patches may appear on any skin surface and patients can feel disfigured.