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Consultant in Special Care Dentistry UCLH/Honorary Senior Lecturer UCL, Royal National ENT and Eastman Dental Hospitals, University College London Hospital, London
Scleroderma is an autoimmune condition of the connective tissue. It encompasses a range of disorders that can affect the face, skin, limbs and internal organs. Common dental manifestations include xerostomia, dysphagia, microstomia, thickened and tight facial skin and tongue rigidity. This article describes how scleroderma can severely impact and compromise oral health. This can occur due to the restricting nature of sclerodactyly and microstomia seen in severe forms of the disease. Dental professionals must be mindful of this condition and its common oral manifestations. Techniques of how to recognize and manage these patients are discussed.
CPD/Clinical Relevance: The dental and oral health implications of scleroderma are relevant to clinical practice.
Article
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.
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