What is juvenile dermatomyositis and its causes?

Juvenile dermatomyositis has no cure. There are a number of drugs that manage to control the acute crisis and in many cases prevent the recurrence of new crises

Learn a little more about juvenile dermatomyositis, a disorder that affects the skin and muscles of the little ones. Fortunately, it is not a common disease.

What is juvenile dermatomyositis?

This is a rare disease that attacks the muscles and skin . A severe inflammatory response of the skin and muscles as well as vasculitis have been observed in biopsies. This leads to systematic muscle weakness, skin problems and certain complications such as calcinosis.

It is given the name “juvenile” because it occurs in children and adolescents under 16 years of age. The average age of onset of symptoms is 7 years.

Juvenile dermatomyositis causes

The Association of Pediatrics states that the cause of this disease has not yet been identified. But there are a couple of theories:

The first is that the pathology originates from a problem with the immune system . It is presumed that an autoimmune mechanism causes damage to the small blood vessels of the skin and muscle, which develops the symptoms.

As a second option, it is presumed that there are microorganisms that trigger the symptoms , such as the coxsackie virus, parvovirus and echovirus, toxoplasmosis or borrelia . According to this theory, infection by these microorganisms can trigger an exaggerated activation of the immune system that ends in damage to small-caliber blood vessels.

No evidence has been found to confirm either theory.

Disease symptoms

The symptoms and discomforts that children with this disease may present are several:

• As general symptoms there may be fever, tiredness, decreased appetite and weight loss.
• There is severe pain in the muscles and skin . Even a light touch can be painful for patients.
• The muscles may feel indurated and enlarged. The most affected are the lumbar, those of the neck and those of the mouth and pharynx.
• The facial skin is inflamed , there is swelling around the eyes, and a butterfly-shaped rash over the cheekbones.
• The rest of the skin on the body may be red, tender, and full of sores .
• There may also be symmetric arthritis of large and small joints and hemorrhages. Respiratory heart disorders and behavioral, memory or learning problems can be added.
• Calcinosis in the skin, muscles and other organs of the body.

Unfortunately all these symptoms are severe. They cause the affected child a lot of pain, limitation and stress.

Diagnosis

The pediatrician will suspect the disease when a child presents skin symptoms accompanied by severe muscle weakness. But he needs to do a long battery of studies that allows him to rule out other immune disorders.

Does juvenile dermatomyositis have a cure?

Juvenile dermatomyositis has no cure . There are a number of drugs that manage to control the acute crisis and in many cases prevent the reappearance of new crises, but they do not resolve it completely. Moreover, there is a high percentage of children who will suffer from a chronic version, regardless of whether or not they have received treatment.

Up to 5% of affected children may develop complications. Such as rheumatoid arthritis, vasculitis, or scleroderma. They can also develop motor disabilities if there are contractures or severe muscle calcinosis.

Juvenile dermatomyositis life prognosis

Approximately 7 to 10% of children with the disease die during the acute phase . Severe muscle weakness ends up affecting the diaphragm and other muscles that promote breathing. The cause of death is respiratory failure.

The rest of the patients may fall into a chronic condition of the disease or suffer some complications.

Treatments

Treatment of this disease is quite complex. Immunosuppressive drugs and others that improve symptoms are needed. Ideally, the patient is evaluated and monitored by a team of several doctors and physical therapists to ensure a good recovery.

The symptomatic crisis can last between 8 months to 2 years.