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Living With Dermatomyositis, A Connective Tissue Disorder

Dermatomyositis is an inflammatory autoimmune connective tissue disorder primarily affecting the skeletal muscles (-myo) and skin (derm-).  It is part of a larger class of disorders known as inflammatory myopathies, which as it sounds, indicates an inflammatory process taking place within the muscle tissue.  When myositis presents with associated skin manifestations, it is known as dermatomyositis, which is recognized as a distinct clinical entity.

While the cause is highly suspected to be autoimmune in nature, the exact mechanism is poorly understood, and may involve a viral trigger.  There is no known cure for dermatomyositis, though recent advances in various therapies regulating the immune response have made it much more manageable than in the past.  Research into the cause (including genetic associations) and available treatments is ongoing.

What are signs and symptoms of dermatomyositis?

There are several characteristic signs and symptoms of dermatomyositis, though not all will be seen in every individual.  Patients with dermatomyositis typically present with a combination of muscular and dermatologic issues, most notably weakness of limb muscles closest to the body frame (proximal muscle weakness) and a red, scaly rash that sometimes itches intensely.

Though these are the hallmark signs and symptoms, there may be additional involvement in other areas, such as the joints, esophagus, lungs and heart, resulting in arthritis, dysphagia (trouble swallowing), dyspnea (shortness of breath) and dysrhythmias (abnormal heart rhythms), respectively.  For this reason dermatomyositis is classified as a systemic autoimmune disease.

It is important to know that despite the name, skin manifestations may often be the only initial indicator of disease; when muscular involvement is noticeable, it may precede, follow or occur simultaneously with skin symptoms.  When muscular problems are evident, the patient often first notices them when he/she has trouble climbing stairs or raising his/her arms to complete daily activities such as brushing hair.

How does dermatomyositis progress over time?

The clinical course of dermatomyositis is quite variable, ranging from moderate weakness and pain to eventual mortality.  For the most part, it is a condition that progresses gradually over time, frequently with periods of remission.

As it progresses, dermatomyositis may involve further muscle weakness/pain and worsening of the characteristic rash, often turning violet/purple in certain locations.  However, thanks to research and better treatment options, dermatomyositis has become a disease quite amenable to successful therapy — and even complete recovery in some cases – i.e., lack of symptoms.

Perhaps most importantly, most cases these days are not life threatening.  There are exceptions of course, mainly occurring in older adults who demonstrate widespread systemic involvement.

Are there other autoimmune conditions that commonly occur with dermatomyositis?

As is frequently the case with suspected autoimmune conditions, it is not uncommon for dermatomyositis to occur alongside other related diseases, such as lupus, rheumatoid arthritis, scleroderma and Sjogren syndrome, among others.

Additionally, dermatomyositis has been associated with an increased risk of certain types of cancer, and some have proposed a connection between dermatomyositis and Lyme disease, though the latter is unproven.

Who gets dermatomyositis?

Both adults and children can develop dermatomyositis, though it is a rare condition regardless of age.  When it is seen in the pediatric population, it is known as juvenile dermatomyositis.  Though anyone may develop dermatomyositis, it is considerably more prevalent in women and the elderly.  It does not appear to have a predilection for a specific ethnicity, but does show possible variation based on region/location.

Questions for your doctor:

  • What are the available therapies for dermatomyositis, and which is best for me (and why)?
  • Can you recommend good sources for the latest comprehensive information on dermatomyositis treatment and research?
  • Do you believe dermatomyositis is associated with viral/bacterial infections?  Why or why not?
  • If I have dermatomyositis, are my children at risk for developing this disorder?
  • Do you know of any clinical trials that are ongoing, in which I might participate?
AutoimmuneMom

About the Author
Dr. Rothbard is a professional medical writer and consultant based in New York City, specializing in medical education articles targeted at a variety of audiences, from children through clinicians.  After leaving medicine, he worked as a biology and medical science educator for several years, before deciding to pursue writing full-time.  He may be reached at grothbard@hotmail.com.

This blog post was originally published by AutoimmuneMom.com, written by Dr. Rothbard, and first published on Apr 10, 2014.

This post contains the opinions of the author. Autoimmune Association is not a medical practice and does not provide medical advice, diagnosis, or treatment. It is your responsibility to seek diagnosis, treatment, and advice from qualified providers based on your condition and particular circumstances. Autoimmune Association does not endorse nor recommend any products, practices, treatment methods, tests, physicians, service providers, procedures, clinical trials, opinions or information available on this website. Your use of the website is subject to our Privacy Policy.

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