Childhood Dermatomyositis

Dermatomyositis is a rare autoimmune disease that causes skin and muscle symptoms. If the disease breaks out before the age of 16, there is talk of childhood dermatomyositis.

Childhood dermatomyositis

Myositis, or inflammatory muscle diseases, belong to the group of generalized connective tissue diseases and autoimmune diseases. Where the immune system of a healthy person defends the body against external pathogens, in autoimmune diseases the functioning of the defense system is disrupted and the body attacks, i.e. forms antibodies against its own tissues. In myositis, this attack occurs against muscle cells. The most well-known myositis are polymyositis or polycystitis and the dermatomyositis of our topic today.

The autoimmune reaction results in inflammation that causes tissue swelling and possibly tissue damage. In childhood dermatomyositis, the inflammation is in the small blood vessels in the skin and muscles. Childhood dermatomyositis is a rare disease that is diagnosed in about four million children each year, slightly more in girls than boys. The disease can break out at any age, but most commonly it occurs when a child is 4 to 10 years old.

Symptoms of childhood dermatomyositis

Childhood dermatomyositis manifests as muscle weakness and pain, especially in the muscles of the neck, shoulders, middle body, and hips. In rare cases, inflammation also occurs in the small blood vessels of other organs. Muscle weakness can cause the child to have difficulty breathing and swallowing problems. Inflammation can also cause swelling and pain in the joints. Because inflammation can be treated fairly easily, they usually do not result in joint damage.

A patient with dermatomyositis often has a rash, especially on the face, eyelids, knuckles, knees, and elbows, in addition to which calcium can accumulate under the skin, forming hard nodules on the skin. Some children also have intestinal symptoms. A sick child is often tired, and fatigue may limit movement and daily activities.

The severity of the disease varies so that some children have only skin symptoms or mild muscle weakness. In more severe cases, the symptoms are on the skin, muscles, joints, lungs and intestines.

Causes of childhood dermatomyositis

About one-third of adult patients have dermatomyositis, but in children, dermatomyositis is not associated with cancer. Experts have not yet been able to determine the exact cause of childhood dermatomyositis, but the current understanding is that it is an autoimmune disease whose onset is influenced by several different factors. Such factors may include, for example, hereditary susceptibility and environmental factors that trigger the disease, such as inflammation or UV radiation. Childhood dermatomyositis is diagnosed by a medical examination, blood tests, and a biopsy or magnetic resonance imaging, for example.

Treatment of childhood dermatomyositis

The method of treatment depends on the severity of the disease and how much it affects the patient’s life. The main purpose of treatment is to reduce the inflammatory state and thus avoid tissue damage. In addition, the treatment aims to restore muscle strength, reduce pain, and improve the child’s overall well-being.

• Medication. Corticosteroids and methotrexates are most commonly used for treatment. Corticosteroids control inflammation effectively and quickly, whereas methotrexate only starts to work after 6-8 weeks of use and is usually a long-term drug. If necessary, your doctor may also prescribe other immunosuppressive drugs.
• Immunoglobulin therapy. In this form of treatment, the patient is given intravenously prepared antibodies isolated from the blood of the donor. Immunoglobulin relieves inflammation by acting on the immune system.

• Physiotherapy and stretching. Physiotherapy helps maintain and improve muscle mobility and strength.
• Food. Calcium and vitamin D supplements are recommended for patients.