Scleroderma is a rare autoimmune disorder which is characterized by hardening of the skin and connective tissue. In other words, the skin becomes tight and patchy. Often this disorder affects the skin of the trunk and limbs. Other symptoms may include digestive problems such as diarrhea, constipation, indigestion, and severe effect on the heart, lungs, and kidneys.
Raynaud’s syndrome is a unique characteristic of scleroderma in which the finger and toes become sensitive to cold temperatures. In severe cases, it can cause bluish discoloration of the fingers and toes associated with tenderness. Although the exact cause of scleroderma is unknown, a defective immune system is considered as the prime suspect to cause scleroderma. Additionally, several genetic and environmental factors may increase the risk of scleroderma.
Every systemic disease has some oral manifestations. Scleroderma can cause tightening of the oral mucosa, restricted mouth opening, limited tongue movement, taste alteration, dry mouth, and increased risk of tooth decay and gum disease. Today’s article will highlight the features of scleroderma. Furthermore, we will discuss the oral manifestations and management of scleroderma.
What is scleroderma?
Scleroderma, also known as systemic sclerosis, is a chronic connective tissue disorder that is typically categorized under autoimmune rheumatic diseases. As the name suggests, ‘sclero’ means hard, and ‘derma’ means skin. (1)
Hardening of the skin is the prime characteristic of this autoimmune disease. Often the symptoms of scleroderma may vary from person to person. However, it is essential to understand that scleroderma is –
- Non-cancerous and non-malignant
The severity of this disease depends on the part of the body affected and the type of treatment delivered.
What causes scleroderma?
Generally, scleroderma is caused by overproduction and accumulation of collagen in the body tissues. Collagen is a type of protein that primarily builds the majority of the connective tissue in the body, including the skin.
Although the exact cause behind the overproduction of collagen is still unknown, it is believed that the immune system of the body plays a crucial role in creating this imbalance. Often a combination of genetics, environmental factors, and immune system disorders may lead to the development of scleroderma. (2)
What are the clinical features of scleroderma?
There is a wide range of symptoms of scleroderma that may affect the health of an individual. Some of them are listed below –
- Skin – hardening of the skin is a hallmark sign of scleroderma. This may often be seen as tightening of the skin and formation of patchy skin. The patches can be formed as ovals or straight lines that cover vast areas of the trunk and limbs.
- Finger and toes – Raynaud’s disease is one of the earliest signs of scleroderma. In this, the small blood vessels of the fingers and toes contract in response to cold temperature or even emotional distress. Often, during this time, the fingers and toes may appear blue and are tender. (3)
- Digestive system – a variety of digestive problems such as heartburn, cramps, bloating, diarrhea, constipation, and difficulty in chewing may be caused by scleroderma. Nutritional deficiency is one of the side-effects of scleroderma.
- Severe cases of scleroderma may affect the functions of the heart, lungs, and kidneys. Such conditions are often life-threatening.
What are the oral manifestations of scleroderma?
Although scleroderma mainly affects the systemic health of an individual, it may also cause oral health problems. Some of the common oral manifestations of scleroderma are listed as follows –
- Microstomia and tightness of the mucosa – in this, patients have a small mouth with tight lining tissue. Often, patients find it difficult to open the mouth thoroughly and maintain proper oral hygiene. Moreover, the adjacent mucosa may pull the gums away from the teeth. Tongue movements may also be restricted. (4)
- Xerostomia or dry mouth may cause discomfort while chewing and swallowing. Lack of saliva also increases the risk of gum disease and tooth decay. Moreover, it can cause taste alteration.
- GERD (Gastro-esophageal reflux disease) can cause chronic throat pain and soreness. Additionally, it increases the risk of enamel erosion and tooth decay.
- Periodontal problems are widespread in patients with scleroderma. (5)
- Hardening of the skin caused by scleroderma often causes myofascial pain and temporomandibular joint pain. This may significantly affect the ability to chew or speak.
- Some other dental effects may include loosening of the teeth, taste alteration, and loss of appetite. (6)
How are oral manifestations of scleroderma treated?
Treatment of the oral effects may depend on the type and severity of the dental problem. Some of the standard management techniques are listed below –
- Exercises of the jaw muscles and lips may help to relieve the tightness and allow adequate mouth opening. A dentist may also use mouth props and manual tools to aid in mouth opening. (7)
- Routine oral hygiene practice such as tooth brushing and flossing is crucial to maintain good oral health and avoid tooth decay or gum disease. Fluoride toothpaste works well in such cases.
- Dryness of the mouth can be treated by increasing the intake of water. Chewing sugar-free gums and candies may help to increase saliva production. Artificial salivary substitutes may help to maintain the moisture. (8)
- Patients with scleroderma are prone to acid reflux. Therefore, it is essential to maintain a proper diet which excludes acidic and sugary food intake.
- Muscle relaxants are excellent for treating myofascial or temporomandibular joint pain.
Working co-operatively with the dental professional will help the patients to recover fast and live a healthier life.
Take away message
Scleroderma is collectively named to a group of autoimmune disorders that primarily cause skin hardening and tightening. In some cases, it also affects the connective tissue. As the definition suggests, it is mainly caused by a defect in the immune system of the body. However, there may be several risk factors that contribute to the increased susceptibility of developing scleroderma.
Systemic scleroderma can cause tightening of the oral mucosa. This results in restricted mouth opening and limited tongue movement. Often the mucosa pulls the gums away from the tooth surface. Poor oral hygiene, dry mouth, taste alteration, acid reflux, and myofascial pain are some of the common oral manifestations of scleroderma. Treatment of dental problems is usually palliative and depends on the type of underlying defect.
It is essential to follow-up your routine dental appointment keep a check on your dental health, especially if you are experiencing the symptoms of scleroderma.