Dermatomyositis in Children (JDM) 4.4/5 (5)

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Dermatomyositis in children

Juvenile dermatmyositis (JDM) is a chronic systemic connective tissue disease in children. Rheumatic inflammation is detected in skin and muscles

Definition

Juvenile dermatomyositis (JDM) is a chronic, systemic connective tissue diseases In children (before the age of 16). Rheumatic inflammation of the skin and muscles results in characteristic symptoms. Special blood tests usually show typical signs of the disease. Dermatomyositis in children has a different disease course than dermatomyositis in adults.

Symptoms of juvenile dermatomyositis

Tiredness and fatigue along with muscle weakness are common symptoms

  • Muscle pain may occur, but not in all cases
  • Thigh muscles are most commonly attacked
    • Symptoms may be trouble getting up from the floor or the chair, climbing stairs and hills
  • Skin symptoms are often typical with red-blue-purple staining and some swelling over the stretch side of the knuckles on the hands (Gottron's sign / papels) and at the extensor side of elbows and knees. Some children get purple upper-eyelid discoloration (Heliotropic eczema)
  • Calcium (calcinosis) under the skin is characteristic, but does not occur in all (10-30%)
  • The areas around the eyes can also swell
  • Some get dry cough and heavy breathing if exerted if the lungs are also attacked
Jevenil Dermatomyosite

Dermatomyosite in 6 year old girl with lime as 0,5-2cm large lumps under the skin and rash. Doh EJ, Ann Dermatol (2016). CC BY-NC 4.0

Diagnosis

Juvenile dermatomyositis (JDM = diagnosed on the basis of symptoms and examination findings.

  • Muscle strength can be tested, often by experienced physiotherapists
  • Blood tests often show elevated CK (muscle enzyme creatine kinase). Some have typical antibodies that include ANA (antinuclear antibody) With special subgroups
  • Muscles are often examined MR which may show signs of inflammation
  • Muscle tissue test (most often from thighs) is applicable to ensure the diagnosis and show the rate of inflammation
  • CT of lungs are done to determine whether the lungs are attacked

Dermatomyositis in children. X-ray shows lime under the skin at the elbow. Boelch SP, Case Rep Orthop (2015). Open

Differential diagnosis

Other muscular diseases with increased CK in children:

Atypical myocyte, fever episodes, elevated CRP over time, livedo reticularis, stroke also consider:

Complications

There is no increased incidence of risk of cancer disease in children with JDM. This distinguishes JDM from dermatomyositis in adults.

Disease Causes

Unknown. Heredity is rare and no infection or triggering environmental factors have been detected.

Occurrence

Very rare disease.

  • One sees only a few new cases in Norway each year

Treatment

Medications for juvenile dermatomyositis are important to fight the rheumatic inflammation initially and to keep the disease under control throughout the course. Often, the inflammation stops within a few years, but treatment and follow-up over several years is necessary.

  • Medicinal treatment with cortisone in the form of Prednisone tablets combined with other immunosuppressive drugs are usually required. The cortison dose may often be high initially, but the dose is gradually reduced. Side effects include weight gain and growth reduction, but are transitory. Other medicines with minor side effects may take over for cortisone later in the course of the course: Methotrexate, Azathioprine and others. Against calcinosis (calcium under the skin) no drugs have shown safe effect.

Diet

No studies indicate that a particular diet or supplements of vitamins, trace elements or otherwise have an effect on juvenile dermatomyositis.

Research

A doctoral thesis (PhD) has been performed at Oslo University Hospital, Rikshospitalet where research is on JDM is continued.

Literature

Children with rheumatic disease, BINDEVEVSSYKDOMMER.no


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