Scleroderma is a chronic systemic autoimmune disease, primarily affecting the skin. The disease is characterized by fibrosis (hardening of the skin). There are two types of scleroderma: systemic scleroderma and localized scleroderma.
This type is characterized by isolated patches of hardened skin. The lesions can appear as ‘stains’ or stripes.
This type is characterized by the involvement of organs, in addition to the skin. This manifestation can cause hypertension, difficulty breathing and more.
Scleroderma is a rare disease with < 3:100,000 incidences annually. The most common form of scleroderma among children is Localized Scleroderma, and it is more likely to affect girls than boys. Only 10% of children affected are diagnosed with Diffuse Systemic Scleroderma.
Scleroderma is an inflammatory disease, but the main cause of the infection is unknown. There is a high likelihood that the disease is an autoimmune disease. This means that your child’s immune system is attacking itself. The infection causes swelling, fever and an overproduction of connective tissue. Until now, there has been no evidence of any genetic link, despite reports of a number of numerous people in one family suffering from the disease. The disease is not contagious.
Limited Systemic Scleroderma is characterized by hardened skin. In some cases, the first stage manifests as a reddish ring ('morphea'). This is indicative of the disease's activity. In later stages, the skin turns brown and white (among Caucasian patients). Among patients who are not Caucasian the lesions look like sores before turning white. The diagnosis is made based on the skin characteristics.
Linear Scleroderma appears as straight lines on the hand and foot, with possible tissue involvement. In some cases, Linear Scleroderma affects the face and skull. Blood tests will show normal results. Linear Scleroderma does not involve the internal organs.
Treatment for Localized Scleroderma
The treatment goal is to stop the progression of the infection as soon as possible. This type of treatment rarely affects the already hardened skin. When the infection is gone, the body absorbs some of the damaged tissue and the skin gets soft again. Drug treatment has proven to be the most effective treatment method. Drugs are prescribed by a pediatric rheumatologist. The progression of the disease usually stops on its own, but if untreated, it takes years to fully recover and the patient may experience recurrences.
Patients with Linear Scleroderma require intensive treatment, including physical therapy. If the affected skin is on a joint, it is important to keep the joint mobile and flexible (massages can help). Using skin creams can help keep the skin soft. Sunscreen is important.
The earliest sign of this disease is a change in the color of your child's hand and/or feet when there is a change in temperature (hot to cold). The skin on these areas in addition to the skin on your child's nose hardens fast and looks shiny. The hardening of the skin can spread to other areas on the body. During the initial stages of the disease, patients are likely to suffer from swollen fingers and pain in their joints. Internal organs are affected as well. Your child's prognosis depends on the type and severity of the organs affected. It is extremely important to monitor the internal organs affected by the disease and to determine their functionality.
There is esophageal involvement among most children suffering from Systemic Scleroderma. The infiltration of stomach acid into the esophagus as a result tends to cause heartburn. Later stages may affect the entire digestive system. Other vital organs that are usually affected include the lungs, heart and kidneys.
Treatment for Systemic Scleroderma
A pediatric rheumatologist will decide on a treatment method for your child. Depending on the organs affected, other medical experts may be involved in the treatment process. There is no drug that has proven to be 100% effective treating Systemic Scleroderma. There are a number of new drugs that are now in the clinical trial stage. Physical therapy helps keep the joints mobile.
Monitoring is needed in order to evaluate the progression of the disease and the effectiveness of the treatment. Due to the involvement of vital organs, it is important to conduct regular tests.
How long does the disease normally last?
Localized Scleroderma generally lasts for a few years. The hardening of the skin usually stops two years after the infection began. Sometimes the infection can last 5-6 years. Systemic Scleroderma is a prolonged illness that can last for a person's entire life. Frostbite can effect a child's growth, whereas the changes in pigmentation are just superficial issues. The extent of the involvement of vital organs varies from patient to patient and also influences the long term effects of the disease. Systemic Scleroderma can be life-threatening.
Children suffering from localized scleroderma can completely recover from the disease. Children who suffer from systemic scleroderma have less of a chance of a full recovery, but their condition can improve and stabilize.