Clinical Spectrum in Juvenile Dermatomyositis

Dermatomyositis is a rare and severe inflammatory disease that affects mainly the skeletal muscle and the skin. Internal organs, such as coronary arteries and the heart, may also be affected. In contrast to the adult form, juvenile dermatomyositis (JDM) is defined as manifestation before the 16th year of life.

For the diagnosis of juvenile dermatomyositis, skin phenomena, livid discolorations of the upper limbs and/or Gottron signs/papules as well as extensor exanthemas are essential. Other criteria include symmetrical muscle weakness, increased muscle enzymes, findings of myositis in MRI examinations and muscle specimen.

Depending on symptoms and clinical course of the disease, mild and moderate forms are distinguished. Depending on the clinical assessment, the appropriate immunosuppressive therapy can be divided into an induction and maintenance phase. The non-drug therapy essentially comprises physiotherapy and otherwise depends on the individual clinical problems. The occurrence of calcinosis is a challenge in therapy. Overall, the aforementioned clinical manifestations and clinical course can be significantly variable and will be demonstrated by different case reports.