Dermatomyositis

Summary

Dermatomyositis is an autoimmune muscle disease that involves inflammation and a skin rash. It is a type of inflammatory myopathy. The cause of dermatomyositis is unknown. Experts think it may be due to a viral infection of the muscles or a problem with the body’s immune system. It may also occur in patients who have cancer in the abdomen, lung, or other parts of the body. Anyone can develop dermatomyositis. It most commonly occurs in children age 5 – 15 and adults age 40 – 60. Women develop this condition more often than men. Symptoms may include: problems swallowing, muscle weakness, stiffness, or soreness, purple color to the upper eyelids, purple-red skin rash, and shortness of breath. The muscle weakness may come on suddenly or develop slowly over weeks or months. You may have trouble raising your arms over your head, getting up from a sitting position, and climbing stairs. The rash may appear on your face, knuckles, neck, shoulders, upper chest, and back. Symptoms may go away completely in some people, such as children. The condition may be fatal in adults due to severe muscle weakness, malnutrition, pneumonia, or lung failure. The major causes of death with this condition are cancer and lung disease.

(Autoimmune Association, 2022)

Degenerative changes of the muscles and skin, caused by an inappropriate autoimmune reaction that leads to obstructive inflammatory changes of blood vessels within muscle, connective tissues of the skin, and other tissues. 
There are three types of DM: 

• Adult dermatomyositis

• Sine myositis

• Juvenile dermatomyositis (JDMS)

(Global Autoimmune Institute, 2023)

Symptoms

The signs and symptoms of dermatomyositis can appear suddenly or develop gradually over time. The most common signs and symptoms include:

• Skin changes. A violet-colored or dusky red rash develops, most commonly on your face and eyelids and on your knuckles, elbows, knees, chest and back. The rash, which can be itchy and painful, is often the first sign of dermatomyositis.

• Muscle weakness. Progressive muscle weakness involves the muscles closest to the trunk, such as those in your hips, thighs, shoulders, upper arms and neck. The weakness affects both the left and right sides of your body, and tends to gradually worsen.

(Autoimmune Association, 2022)

Muscle weakness, stiffness and soreness, difficulties in swallowing (dysphagia) and articulating speech (dysphonia), reddish-purple rash (heliotrope) present in eyelids, on forehead, cheeks, and bridge of the nose, scaly regions of skin, a “dusky” rash which is itchy, fatigue, and weight loss.
Symptoms specific to each of the three types of the DM: 

• adult dermatomyositis: low-grade fever, lung inflammation, and sensitivity to light

• sine myositis: skin abnormalities may occur without associated muscle abnormalities 

• juvenile dermatomyositis (JDMS): Similar symptoms to the adult form but with a more sudden onset. Abnormal accumulations of calcium deposits in muscle and skin tissues and GI tract involvement are more common here.

(Global Autoimmune Institute, 2023)

Diagnostic Criteria

Under Investigation

Study Classification Criteria

Under Investigation

Diagnostic Tests

Under Investigation

Organized Autoimmunity

(Alternative Autoimmune Disease Classification: FIEM, MIEM or BIEM, or FEM, MEM or BEM)

1. sex predominance (is an autoimmune disease primarily found in genetic Females, Males, or equally in Both?)

   1. Under Investigation

2. Inherited and acquired gene variations that cause increased susceptibility

   1. Human Leukocyte Antigen (HLA) Associations

      1. Under Investigation

   2. Other Gene Variations (mutations)

      1. Under Investigation

3. Gene Triggering Environmental Exposures

   1. Infections

      1. COVID-19 (Chang et. al, 2023, found a significantly higher risk of “Dermatopolymyositis” following COVID-19 infection in a study of 3,814,479 participants)

         1. {What is the diagnostic difference between Dermatopolymyositis, Dermatomyositis and Polymyositis?}

   2. Toxins

      1. Under Investigation

   3. Stress

      1. Needs to be assessed for each patient

4. Multiple interactive and destructive immune system pathologies

   1. Under Investigation

Tissue-Type or Cell-Type Attacked

Under Investigation

Treatment(s)

Intravenous Immune Globulin has shows efficacy, with side effects (Aggarwal et. al, 2022).

Managing Specialist(s)

Under Investigation

Research Authors

Under Investigation

Research Institutions

Under Investigation

Average Time from Symptom Onset to Diagnosis

Under Investigation

Last Updated

September 05, 2023

References

Chang R, Yen-Ting Chen T, Wang SI, Hung YM, Chen HY, Wei CJ. Risk of autoimmune diseases in patients with COVID-19: A retrospective cohort study. EClinicalMedicine. 2023 Feb;56:101783. doi: 10.1016/j.eclinm.2022.101783. Epub 2023 Jan 10. PMID: 36643619; PMCID: PMC9830133.

Dermatomyositis. Autoimmune Association. (2022, November 09). Retrieved January 05, 2023 from https://autoimmune.org/disease-information/dermatomyositis/

Dermatomyositis. Global Autoimmune Institute. (2023). Retrieved January 05, 2023 from https://www.autoimmuneinstitute.org/dermatomyositis/

Rohit Aggarwal, M.D., Christina Charles-Schoeman, M.D., Joachim Schessl, M.D., Zsuzsanna Bata-Csörgő, M.D., Dr.Sc., Mazen M. Dimachkie, M.D., Zoltan Griger, M.D., Ph.D., Sergey Moiseev, M.D., Chester Oddis, M.D., Elena Schiopu, M.D., Jiri Vencovský, M.D., Dr.Sc., Irene Beckmann, Elisabeth Clodi, Ph.D., et al., for the ProDERM Trial Group. Trial of Intravenous Immune Globulin in Dermatomyositis. October 6, 2022 N Engl J Med 2022; 387:1264-1278. DOI: 10.1056/NEJMoa2117912. https://www.nejm.org/doi/full/10.1056/NEJMoa2117912query=recirc_curatedRelated_article