Transcription:
Muscle Weakness and Rashes in Kids: Could It Be Dermatomyositis?

Melanie Cole (Host):  If your child is suffering from muscle weakness and an unexplained rash, dermatomiositis could be the cause. My guest today is Dr. Araat Patel. He’s a board certified specialist in Pediatric Rheumatology, Pediatrics in Internal Medicine at UVA Children’s Hospital. Welcome to the show, Dr. Patel. Tell us a little about muscle weakness and unexplained rash and what would send the parent to the doctor. How do you diagnose this? 

Dr. Araat Patel (Guest):  Thank you for having me on the show. Well, dermatomiositis is an inflammatory disease of the muscle, skin, and blood vessel. The symptoms are very nonspecific, but for someone who’s seen many patients with dermatomiositis, usually an adult rheumatologist or pediatric rheumatologist, it’s fairly easy. But for someone such as a pediatrician who has to see diseases from a vast majority of specialties, it’s a little difficult because of the nonspecific symptoms. Once we see a patient with that classic rash, we usually know what the diagnosis is. 

Melanie:  So do we know the causes, you know why. Is this an autoimmune disease? 

Dr. Patel:  Right. So we do not know the cause, but it is an autoimmune disease, meaning your immune system is revved up and attacking parts your body that it should not.  

Melanie:  So what groups of children are most likely to be affected by that? 

Dr. Patel:  So all ages of groups, all ethnics groups, but most cases in children occur between ages of 5 and 10 and about twice as often in female. 

Melanie:  Is it hereditary, Dr. Patel? 

Dr. Patel:  It is not hereditary, but a lot of the autoimmune diseases, such as lupus, rheumatoid arthritis, dermatomiositis come in batches. So there’ll be a family history of another autoimmune disease, possibly. 

Melanie:  So how scary for a parent, especially when there’s muscle weakness involved -- a parent’s mind goes in all different directions. Once they’ve come to see you and you have diagnosed this, what treatments are available, and is this something that the child will have to live with now for the rest of their life?  

Dr. Patel:  So we say that you will have this for the rest of your life, and our goal of treatment is remission. And we can achieve remission fairly quickly. We usually use corticosteroids, which are prednisone, Solu-Medrol. We use that for a brief period of time, and then we use the same medications that we use for rheumatoid arthritis. And one of them is methotrexate, and another medication for those children that are little more sick is something that we give in the hospital through an IV for about 24 hours every month called IVIG. The treatments are really good, and we can control the majority of cases and put them into remission, and they stay on medication for a few years, and then sometimes they come off of medication. So technically they’re cured, but we never say that they’re actually cured, just in case they flare up in the future. 

Melanie:  What would happen if this is left untreated?  

Dr. Patel:  Usually the child would get very sick, and it will start affecting the muscles which control breathing and swallowing. So they will end up in some sort of emergency room, urgent care center, or sometimes even in intensive care unit because they can’t breathe or can’t swallow, and their secretion, their spit and their food is going down the wrong tube, into their lungs. So usually they’ll end up sick and someone will figure out the diagnosis.  

Melanie:  For some children, Dr. Patel, is there a need for physical therapy? What’s their daily life like? And can they mainstream in school, or is this something that’s going to affect their normal living? 

Dr. Patel:  So it does depend on the severity. Once we diagnose it, we are very aggressive with treatment. We try to get their muscles functioning normal as soon as possible. They may have already suffered some muscle atrophy, so physical therapy is very important. Sometimes we send them to aquatic therapy in a pool; sometimes we send them to physical therapy in an inpatient unit, depending how sick they are. But once we start treating them, they usually will live a normal life with some restrictions, such us the sun. So, where I live in Virginia, it’s pretty sunny, so some of these children have to make sure they apply sun block on every day because that could make their disease flare. The majority them live a normal life as long as we can control the disease. 

Melanie:  Now you mentioned steroids and the methotrexate. These medications begin to work, and then the parents and the child will see some results. They’ll see them getting better. Do they stay on steroids, and does that have its own side effects? 

Dr. Patel:  So steroids have a lot of side effects so we try not to use as much steroids as some of the pediatric rheumatologists 20, 30, 40 years ago had to because they didn’t have some of the good medications we have now. So I try to keep patients on steroids for a minimum of one month and a maximum of maybe three months, if anything, depending on how bad their disease is. And then the other medication such us methotrexate, IVIG, Imuran, they sound scary when you read the prescribing information, but in fact they’re very well tolerated and even better tolerated in children compared to adults. 

Melanie:  Are there any comorbidities that go with this? Is there anything that parents should look out for as the child approaches their teenage years and on? Is this going to put them at risk for other autoimmune diseases—lupus or Crohn’s or MS or any of these others? 

Dr. Patel:  So once you have one autoimmune disease, you have a higher risk of acquiring another autoimmune disease, such as thyroid disease, psoriasis, lupus. But usually they’ll just have this one disease. The comorbidities come in from the disease severity. So, some of these children, especially teenagers, when they can’t keep up with their peers, there is sometimes depression, sometimes anxiety. Other things are occurring at the same time in their life that should be occurring normally in a child’s life. So, the multidisciplinary approach is usually physical therapy, sometimes psychology, sometimes a counselor just to get them through feeling abnormal because they will probably be the only person with this disease because of how rare it is. 

Melanie:  So what about dietary needs? If chewing or swallowing or muscular weakness can be an issue, are there certain foods you like to recommend to parents that they feed this child? Is it going to affect their eating habits for a long time? 

Dr. Patel:  Not for a long time, only in the beginning. If they truly have some problems swallowing, we are aggressive with the steroid dose, and we usually try to fix that in a few days to weeks. So maybe for a few days, you can’t go to a steakhouse and eat some hearty meat that gives you trouble swallowing. But most of the time there are no foods that are going to make this better and there are no foods to really avoid, just the normal foods that everyone should avoid, such as processed food and fast foods, but nothing out of the ordinary. 

Melanie:  So in just the last few minutes, Dr. Patel, if you would, give your listeners best advice and why they should come to UVA Children’s Hospital for their care. 

Dr. Patel:  So like I said, unfortunately, dermatomiositis can cause nonspecific lab abnormalities, nonspecific symptoms. So if you are in a position where you’re going from one specialist to another specialist to another specialist, sometimes you have to think about rheumatology—“maybe I should go see a rheumatologist”—especially if there’s fever and rashes, muscle weakness and joint pain and joint swelling. So at UVA, we use a multidisciplinary approach. We are aggressive with our treatments, and we try to get these children back on their feet. We have excellent physical therapy there. We have excellent ancillary services, and most of the families in Virginia are very happy when they come to UVA for their care. 

Melanie:  Thank you so much. It’s really great information. You’re listening to UVA Health Systems Radio. For more information, you can go to uvahealth.com. That’s uvahealth.com. This is Melanie Cole. Thanks so much for listening.