Dermatomyositis is a rheumatic disease that attacks the muscles, weakening them, as well as the skin, which manifests itself in various, characteristic changes. What are the causes and symptoms of dermatomyositis? What is the treatment?

Dermatomyositis(Latindermatomyositis , abbreviated as DM) is a type of polymyositis - a rheumatic (specifically systemic connective tissue), in the course of which chronic inflammation in the muscles occurs. In patients with dermatomyositis, the skin is also affected. Only in rare cases does the muscle not become involved (Latindermatomyositis sine myositis ).

In adults, dermatomyositis affects 5-10 people out of 1 million, and in children - it affects 1-3 people out of 1 million.

Dermatomyositis - causes

The exact causes of dermatomyositis are unknown. It is suspected to be an autoimmune disease, i.e. one in which the cells of the immune system attack the body - in this case the muscles and skin.

GOOD TO KNOW>>Disorders resulting from autoimmunity, i.e. AUTOIMMUNOLOGICAL DISEASES

Some people suspect that viral, bacterial or parasitic infections may be factors that trigger an abnormal immune response. In turn, in people who have a predisposition to the development of the disease, exercise, ultraviolet radiation and medications.

Dermatomyositis - symptoms

The first symptoms of dermatomyositis are malaise, fever and general weakness. Then the skin changes appear:

  • erythema of the skin of the neckline and neck (V-sign)
  • eyeglass-shaped erythema and swelling around the eyes (so-called pseudo-glasses, symptom of heliotrope)
  • erythema on the nape, neck and shoulders (the so-called shawl symptom)
  • erythema on the side of thighs and hips (the so-called holster symptom)
  • Gottron's symptom, i.e. lumps, erythema and widening of small blood vessels on the backs of hands and over small joints
  • Keining's symptom, i.e. erythema and ecchymosis within the nail folds. Then the accompanying symptom is often tenderness andsoreness of the nail shafts under the pressure of the so-called mechanic's hand, i.e. hard and coarse skin of the fingertips and hands, prone to cracks and peeling

So-called pseudo glasses and Gottron's lumps are the most characteristic symptoms of dermatomyositis

Some of them may also appear, inter alia, red follicle dandruff, diffuse alopecia or body hair (hypertrichosis).

In turn, children have a lilac color of the entire face (lilac disease), and often there are also calcium deposits in the subcutaneous tissue, most often around the joints.

CHECK>>Bruises, rash, spots ON SKIN - what diseases reveal

In addition, similarly to polymyositis, it also happens:

  • muscle weakness - especially in the arms and thighs (which results in problems with combing and climbing stairs), as well as the neck muscles (which may lead to the head falling inertly) or the esophagus (then swallowing, breathing or speech)
  • joint pains and morning stiffness

In contrast, myocarditis, cardiomyopathy, arrhythmias or pulmonary hypertension develop less frequently than in the case of polymyositis.

Read also: Rheumatic profile - tests for rheumatic diseases

Important

Dermatomyositis and cancer

In patients with dermatomyositis, the risk of malignant neoplasm of the internal organ is 20-25%. The most commonly diagnosed accompanying neoplasms are ovarian, lung, gastrointestinal, breast and non-Hodgkin's lymphomas. Most cancers are diagnosed within one year of diagnosis of dermatomyositis, but some say the cancer may appear up to 5 years after diagnosis of DM. Therefore, the patient should be under oncological supervision.

In turn, other researchers have demonstrated increased cancer detection before the onset of dermatomyositis symptoms (up to 2 years before the onset of DM) and suggested that in some patients the disease may be a paraneoplastic syndrome, i.e. a set of lesions that give characteristic symptoms that may testify to the coexistence of the neoplastic process.

Read also: Fibromyalgia (FMS) - a disease manifested by pain in muscles and bones

Dermatomyositis - diagnosis

If dermatomyositis is suspected:

  • blood tests - with DM, there is an elevated level of condition parametersinflammation (including Biernacki's reaction), as well as muscle enzymes in the blood (CK, aldolase, transaminases)
  • electromyographic examination (EMG) - shows muscle damage
  • biopsy of an altered muscle, usually deltoid muscle, which shows inflammation of the muscles

Dermatomyositis - treatment

The patient is given immunosuppressive drugs (usually prednisone), which "silences" the immune system, and thus the inflammatory process in the body. Over time, the patient is given smaller and smaller doses of the drug.

If after 4-6 weeks there is no improvement or the symptoms of the disease worsen after reducing the dose of prednisone, other immunosuppressants are added to it - a choice of methotrexate, azathioprine, cyclophosphamide.

If the above-mentioned the therapy does not bring results, intravenous immunoglobulin preparations are administered for 3-8 months.

Monika Majewska A journalist specializing in he alth issues, especially in the areas of medicine, he alth protection and he althy eating. Author of news, guides, interviews with experts and reports. Participant of the largest Polish National Medical Conference "Polish woman in Europe", organized by the "Journalists for He alth" Association, as well as specialist workshops and seminars for journalists organized by the Association.

Read more articles by this author

Category:

Spine, bones, joints