The dermatomyositis It is one of the autoimmune diseases classified as rare because it only affects between 4 and 6 people per million inhabitants, and it affects the skin and muscle tissue.
Approximately 20% of all dermatomyositis They debut in pediatric age. , since both children and adolescents can develop juvenile dermatomyositis (DMJ), with a peak incidence between 5 and 14 years.
On the National Day of Juvenile Dermatomyositis, which is celebrated this Tuesday, April 23, the ANADEJU Association and the Spanish Society of Rheumatology insist on the need to increase the visibility of this disease, to carry out more research projects of the disease and guaranteeing multidisciplinary care.
Inflammation in the skin and muscles
The development of the disease involves inflammation of the skin and muscles caused by the patient's own immune response, causing skin lesions or muscle weakness.
It is a chronic pathology that occurs with outbreaks, which has a variable intensity, and which, according to the Dr. Alina Boteanu, spokesperson for the Spanish Society of Rheumatology (SER) and the Rheumatology Service and the Pediatric Rheumatology and Transition Unit Hospital Universitario Ramón y Cajal (Madrid).
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“Close and regular follow-up in rheumatology consultations is necessary to maintain adequate control of the disease.”
Main symptoms of dermatomyositis
The main symptoms suffered by people with this rare disease are:
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Pain
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Fatigue
Difficulties in doing physical exercise appear and, in later stages, the patient may have problems performing routine activities such as walking long distances, playing or climbing stairs.
In some children, muscle contractures or joint inflammation may appear, which can cause pain or difficulty in certain movements.
So much for the muscular part of the dermatomyositis.
Where do dermatomyositis skin lesions appear?
Well, if we focus on the skin problemsskin lesions are also common and can appear even before muscle inflammation.
Habitually They affect areas such as the face, neckline and handsbut they can be seen anywhere on the body.
Furthermore, as explained by the Spanish Rheumatology Foundation, these injuries They are different depending on the place in which they appear:
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Dealing with the expensivethere usually appears a “purplish rash surrounding the eyes”. This lesion can also appear in the oiliest areas of the face.
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In the region cervico-dorsal appears what experts call «shawl sign” either “capeline erythema», which is nothing other than a redness of the skin that spreads along the neck.
- If it affects the handss, the lesions typical of dermatomyositis are identified by the appearance of plaques. And violet plaque-like lesions that peel off, similar to those that appear with psoriasis, can be identified.
On some occasions, few according to experts, these skin manifestations can be complicated by appearing calcinosis (appearance of nodules) or lipodystrophy (problems in the body's disposal of fat).
JDM is a rare disease, with an estimated incidence of 4 cases per million children per year. Children who suffer from this pathology may find themselves tired and have difficulty doing physical exercise; even in later stages they may have difficulty performing some common activities of daily living such as walking longer distances, playing or climbing stairs. In some children, muscle contractures or joint inflammation may appear, which can cause pain or difficulty in certain movements.
Skin lesions may appear before muscle inflammation. It usually affects regions such as the face, neckline and hands, but it can be seen anywhere on the body. In rare cases, complications such as calcinosis or lipodystrophy may appear. Other manifestations such as pulmonary or cardiac involvement are observed in a small number of children.
How serious is this disease?
Furthermore, dermatomyositis Rarely, it can affect the lungs or kidneys. heart.
And as Dr. Boteanu details:
- “The severity of the disease is very variable, from very mild skin lesions without muscle involvement to involvement of several organs in more serious cases.
The clinical manifestations of dermatomyositis They are similar in children and adults, but the frequency of appearance is different. For example, calcinosis is more common in children and, furthermore, in pediatric age this pathology is not associated with neoplasms.
However, the specialist insists that:
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“The development of various fdrugs and the increase in therapeutic options along with a diagnosis and a early treatment have led to a significant improvement in prognosis in recent years.
In this sense, “most patients that achieve adequate control of the clinic, They remain asymptomatic. On the other hand, if the disease is not controlled, damage can occur and this can be irreversible,” warns the expert.
Pharmacological and physiotherapy treatment
Drugs used to control inflammation are immunosuppressive medications that act by inhibiting certain pathways of immunity.
Among the most frequently used medications are: glucocorticoidsthe immunoglobulinsother classic or biological disease-modifying drugs, as indicated by the specialist.
On the other hand, in recent decades it has been considered rehabilitation/physiotherapy as “one of the pillars of the non-pharmacological treatment of dermatomyositis“.
Several studies have shown that supervised and targeted rehabilitation exercises are beneficial for mobility, strength and muscle mass without worsening inflammation.
“Contractures and reduced mobility can be relieved with rehabilitation sessionsHowever, it is important that the procedures are carried out in a controlled manner in the early stages of the disease,” says the Dr. Boteanu.
