In recent years, much research has focused on understanding dermatomyositis and developing new treatments for this challenging disease. We now understand, for example, that not all DM patients experience muscle symptoms. And a number of clinical trials are currently in process, testing new medications that show promise in treating DM.
TMA medical advisor and dermatologist Dr. Ruth Ann Vleugels specializes in myositis skin disease, including amyopathic dermatomyositis (ADM). She serves as director of autoimmune skin diseases and is an associate professor of dermatology at Harvard Medical School.
Dr. Vleugels will respond to members’ questions related to skin symptoms in DM and ADM.Ask a Question
My medication are tacrolimus for hands and ketoconazole for scalp involvement. Are there more effective medications? I have flares frequently. I wear hats and gloves as well as sunscreen on my hands.Dr. Ruth Ann Vleugels:
There are more effective therapies.
Topical steroids will work better than the ones you are on or at least in conjunction with these.
We also have many systemic therapies i you are interested in pills/infusions (Plaquenil, methotrexate, CellCept, IVIg, etc).
Is myositis an autoimmune disorder?Participant:
So many times, you hear about warnings for people "with compromised immune systems." Do we belong in this group if one has DM or s-IBM myositis?Dr. Ruth Ann Vleugels:
Myositis is an autoimmune disease.
I consider my DM patients to have a compromised immune system if they are on immunosuppressive therapy specifically.
How much/how extensive of a rash should I "tolerate"?Participant:
Since so many of the drugs used to treat DM have significant side effects, what is the best balance to try to achieve between having completely clear skin versus tolerating some level of rashes? Is it better to take fewer meds (or taking some meds at a lower dosage) and deal with fewer side effects, versus treating the skin disease aggressively and being left to cope with problematic medication side effects? Also, does the presence of skin disease correlate with systemic inflammation (a little rash means inflammation in body is not under control)?Dr. Ruth Ann Vleugels:
This is a frequent discussion I have with my patients. As above, for skin specifically, we really can make this decision in conjunction with our patients and their quality of life. We try to choose medications that have the fewest side effects for our patients. In adults, we can choose with each patient how aggressive they would like to be.
There is a different way of thinking in children with this disease. Most children also have muscle disease, and if the skin is active (including the nailfolds), we continue to treat to try to bring them into full remission with no rash and no activity along their nails.
I am currently in the middle of the worst flare I’ve ever had, mostly on my face. I am using a topical steroid and oral steroids, with CellCept soon to be added if necessary. My question is, will my skin ever get back to normal? It’s currently dark red and raised up with grooves in it. What might I do to improve this?Dr. Ruth Ann Vleugels:
- It is critical to wear a wide-brimmed hat. It’s the best thing to keep the pigment change from becoming permanent use
- Sun protection.
- You can add topical tacrolimus in the breaks from topical steroids.
- Consider adding CellCept, so you can get off of the oral steroids and to prevent flares in the future.
Is how the nailfolds look (capillary drop out, inflammation, etc.) a "sentinel" for whether or not the disease is under control?Participant:
If this true why don't more doctors use capillaroscopy to follow how patients are doing?Dr. Ruth Ann Vleugels:
MDs who are trained in nailfold capillaroscopy use this regularly, and we are trying to train all young MDs to use this. This is most important in children in terms of muscle plus skin disease, but we also watch them in skin disease in adults.
Refractory DM—initially minimally myopathic DM with skin component predominant along with livedo reticularis and Raynaud’sParticipant:
Hello. I am a physician with an 18-month history of refractory DM. It began suddenly during a bout of diverticulitis. I was stable using Plaquenil 300mg/dau, CellCept 3gm/day, IVIg 1gm/kg/month for one year, but recently I experienced new onset of dysphagia, vocal weakness, and dyspnea. These symptoms have improved after one month on 1mg/kg/day of prednisone. I am not tolerant of methotrexate. Any thoughts about rituximab? All myomarkers, EMG normal, malignancy work up negative. Any ideas would be appreciated.Dr. Ruth Ann Vleugels:
- If all blood levels of muscle markers are normal, we often do an MRI of the bilateral thighs (myositis protocol) to determine whether the muscles are active.In your case, dysphagia and other symptoms are signs of significant muscle involvement, so I assume that they are not even questioning this.
- For refractory muscle disease, I would add rituximab as an option; in severe dysphagia, we even do pulse steroids (IV, larger doses) to bring people under control more quickly as well.
- I hope you are seeing a myositis expert, given your dysphagia and dyspnea.
- If your PFTs or high-resolution chest CT has changed, you would typically get rituximab, tacrolimus, or even Cytoxan for pulmpmary disease not adequately controlled on
I'm an IBM patient. I have crusty blotches on my scalp, forearm, and top of my hands.Participant:
The blotches crack and split and bleed, and skin cells flaky like dandruff.Dr. Ruth Ann Vleugels:
I do not think that this is related to your IBM, but I would see a good dermatologist to see if the scalp and/or hands can be treated.
Any suggestions for severe body itching?Participant:
My body itches every day. There is what seems a traveling severe itching pattern that is worse than the regular itching. Sometimes there is a rash, most times there isn’t. This is constant.Dr. Ruth Ann Vleugels:
If your itch is over the entire body, not just in areas of the DM rash, we call this “generalized pruritus,” and it needs a work-up with a doctor to ensure some blood levels are okay (and often a chest x-ray). If it is DM-related itch, then we know we are not adequately controlling your skin and need to get you on a better regimen.
If all of this has been done, we can use nerve-directed itch medications (like gabapentin and pregabalin) to try to help itch, which is very challenging for our patients.
Thank you for your time. I am currently on IVIg, which is taking care of the skin rashes and most of the facial swelling. Unfortunately, approximately 1 1/12 to 2 weeks prior to the next treatment, my eyes begin to swell and the itching begins all over. But most troublesome is the fatigue, joint pain, and muscle aches. What else do you suggest?Dr. Ruth Ann Vleugels:
1) Typical treatments with IVIg are two infusions on two adjacent days four weeks apart.
2) If that is how you are getting IVIg, then you may need another agent to help you achieve better control, or you may need better sun protection.
3) If you are getting your IVIg less often, you may need it as described above.
Refractory DM - holistic therapiesParticipant:
Hi. Do you have any success with "anti-inflammatory" diets, meditation, or any other alternative treatments? Thanks.Dr. Ruth Ann Vleugels:
Please see my previous response. I do not think that any particular diet itself is extremely helpful, but good sleep and stress reduction is helpful.
My left side (upper arms and upper legs) is much weaker than the right side. Any reason?Participant:
My left side (upper arms and upper legs) is much weaker than the right side? Any reason?Dr. Ruth Ann Vleugels:
This is not typical of DM and should be addressed by an expert rheumatologist or neuromuscular physician.
ADM mild skin disease questionParticipant:
If I am only experiencing minor skin symptoms while on Plaquenil, is it best to just treat with topicals? Is it harmful in any way to still have cuticle issues and occasional minor rash if all other lab work is good?Dr. Ruth Ann Vleugels:
If a patient has only skin disease (no muscle disease, no lung disease, etc.), we can treat them based on how much the rash impacts their quality of life. We do not have to treat their mild skin disease at all if it is not bothersome to them. It is not harmful to the patient. (This is a very different concept than in patients with muscle or lung disease, however.)
Therefore, we treat with the level of therapy that best clears the rash to the desire of the patient, depending on how itchy they are, how much the rash bothers them in terms of the appearance, etc.
Duration of topical steroid and topical Protopic (tacrolimus) treatmentsParticipant:
Hello Professor Vleugels. I am a 63-year-old male and relatively new DM patient, having first had skin symptoms on my hands about six months ago. I remain amyopathic, which I am exceptionally grateful for. My dermatologist has me on a rotating five days Protopic, two days steroid (both twice/day), and now I am on 300 mg/day Plaquenil (last four weeks). My symptoms have improved about maybe 75%. I have two questions: (1) will I likely stay on this topical treatment and Plaquenil for the rest of my life? And (2) am I likely to remain amyopathic? Thank you.Dr. Ruth Ann Vleugels:
1) It sounds like you are doing fairly well on very mild treatments, which is good news. If you would like to get your skin even more clear/less itchy, you may need more aggressive medication (but you do not need to do this if you skin is not bothering you enough to want to do that).
2) You will likely stay on these therapies for some number of years, we just cannot predict how many years. Most patients do experience disease remission at some point, but that can be four years or 20+ years.
3) If you have had disease for six months or more, you are quite likely to remain “amyopathic.” Once you hit the two-year mark, we essentially think you have almost zero risk of getting muscle disease.
DM with associated lipoatrophyParticipant:
I have DM with associated lipoatrophy. My skin rash is horrible all over my body. In the last four months, it has attacked my fat cells. It is truly a nightmare, leaving my face and shoulders looking like a skeleton. Are you familiar with this?Dr. Ruth Ann Vleugels:
This is a rare complication, usually seen in children with dermatomyositis, but I have seen it in adults as well. If a patient is developing new areas of lipoatrophy, I try to determine whether their disease is adequately controlled.
I have noticed when I have been on antibiotics for things like pneumonia,
And infections. That my skin has cleared up and I have felt better.
Is there any evidence or useage of antibiotics helping DM?Dr. Ruth Ann Vleugels:
Some antibiotics have good anti-inflammatory properties. Anti-inflammatory medications can help the skin rash, which is why you may notice this. This is not something we always expect to see, but I have heard this from some patients.
Avoiding sun exposureParticipant:
Are the effects of UV-A and UV-B systemic, or local? That is, if my rash is confined to my hands, is it important to use sunscreen on all exposed parts of my body, or just my hands?Dr. Ruth Ann Vleugels:
Some patients with dermatomyositis who get sun exposure on their skin can have a flare of their muscle, that is, the inside disease. This is much better described in a disease called lupus, but it has been described in dermatomyositis as well. It is most important to cover all areas that have rash or tend to be affected by the typical dermatomyositis rash, but this usually means covering all areas except the knees down. Keep in mind some patients still get rash on their lower legs.
Links to other autoimmuneParticipant:
I have immune thrombocytopenia (ITP), psoriasis, and DM. Has there been any link between any of these as precursors for each other, and is it common for a person to be susceptible to so many autoimmune issues. Thanks for your time in advance.Dr. Ruth Ann Vleugels:
Many of our patients have multiple autoimmune conditions, so you are not alone! I have many patients with three, four, or even more autoimmune conditions. These tend to be patients who have a strong genetic predisposition for autoimmunity in their body.
Which sunscreens are the most effective? Is it okay to use sunscreen past its expiration date?Dr. Ruth Ann Vleugels:
The best sunscreens have a physical blocker like zinc or titanium in them.
Blue Lizard for sensitive skin
It is not ideal to use them very far past the expiration.
Also, you should apply one golf-ball-size amount of sunscreen to your body to get the protection that is labeled on the bottle. Most people do not use enough sunscreen.
Spray sunscreens tend not to be as effective as creams.
Also, as above, sunscreens should be used in conjunction with wide-brimmed hats and sun-protective shirts, which offer even better protection.
My dermatomyositis patients also wear gloves in the car while driving to protect their hands from the sun. Some buy sun-protective gloves online.
How can a DM patient cope with sun (besides SPF sunscreen)?Participant:
I am a 40-year-old woman with DM (diagnosed in 2013), now taking prednisone 2.5mg/day and methotrexate 20mg/week. Muscular complications are under control, but skin is still in rash and itchy, with classic DM skin signs (heliotrope, Gottron papules...) plus high sensitivity to sun. Since I live in a very warm and sunny state, coping with all the skin problems is highly difficult. Even using 50 SPF—plus thick clothes with long sleeves, hat, umbrella—skin reacts very badly to sun, even to sun light. Is there a solution to that? How can I improve the effectiveness of sun blocking? Thank you very much!Dr. Ruth Ann Vleugels:
Two considerations would be:
1) Taking Plaquenil as a medication helps with photoprotection itself—even if your skin is doing well, it helps with sun sensitivity. So this is a consideration. (We use it for many photosensitive skin rashes.)
2) Sun protective clothing and wide brimmed hats are the best option. We consider sunscreen with a physical blocker—zinc or titanium—as helpful to add to that, but you always want to be wearing a wide-brimmed hat and sun shirt as you mention.
3) Your rash sounds like it could be improved more with medication. One medicine I would consider would be IVIg, which tends to work well for skin disease. CellCept would also be reasonable. There are also other options.
Why doesn’t current dermatological treatments work for ADM?Participant:
It seems clear that many patients (including me) have ADM that is recalcitrant to immunosuppressants. The only thing that works for my rashes is IVIg. Why do dermatologists and rheumatologists as well as insurance companies still insist on making people like me go through a year of prednisone, Plaquenil, CellCept, methotrexate, immunosuppressants, and ointments when none of these do anything and further complicate the illness and possibly increasing the risk of progressing to muscle involvement?
I’m sorry to say, ALL my dermatology appointments were useless besides the couple of biopsies, which were not definitive either.
It seems to me that the treatment for ADM has to be rethought.
One more question is, why there is so little talk about causes of autoimmune issues, such as environment, nutrition, gluten, dairy, and inflammation? It seems Western medicine is failing us patients.Dr. Ruth Ann Vleugels:
You are correct: skin disease is more challenging than even the muscle disease in many patients. IVIg can be the best treatment for many patients.
Seeing a dermatologist with expertise in this area is critical. Depending on where you live, we can help you with this.
We are developing a new drug for the purpose of treating skin disease specifically. It is now in a randomized, controlled trial, so we hope that this will work and then be an option in the future.
Researchers are still trying to find the cause or multiple causes of autoimmune disease. We have not sorted this out completely yet, which is why there is not so much discussion of this in the mainstream. Our best knowledge is that there is a genetic predisposition with a secondary trigger to the body, which could be a virus, etc. We are working to learn more about this to better help our patients.
Managing very swollen base of fingernails (cuticle area)Participant:
I was diagnosed with classic DM. The base of all ten fingernails (cuticle area) are constantly very swollen. I use lotions to try to keep these areas from cracking. Sometimes they become yellow and have pus. It is painful. I've been on numerous medications with no luck. What OTC hand lotions do you believe could work best? I use "Working Hands" and Gold Bond. Thanks!Dr. Ruth Ann Vleugels:
What you are describing is very typical for “activity” in skin dermatomyositis, so it is indicative that your rash is still active. If this is bothersome to you, it requires therapy directed at the dermatomyositis itself. If you are not interested in therapies that are pills/infusions, we would usually use a potent topical steroid around this area under Band-Aids at night (ex: betamethasone dipropionate ointment). Apply a thick layer like frosting and then cover with a Band-Aid.
If this is not working, it means you would need systemic therapy for this problem, that is, pills, infusions, etc. When your skin disease is better controlled, this will improve as well.
I have ADM. Is there a medication coming soon for skin rash? Are there any studies?Participant:
I have the ADM with the rash only. Is a medication coming soon? Are there any clinical available in the southwest, mainly Phoenix area?Dr. Ruth Ann Vleugels:
We do have a current randomized, controlled trial specifically for skin disease in dermatomyositis that has recently started at 12 sites in the US. One is in Arizona. TMA has information on this trial. Even if you are not eligible for this trial (there are always criteria about who is able to participate in a trial), it is good news that we finally have a trial for skin disease, and we hope that there will be more on the way. There are other trials that are looking at both skin and muscle disease as well.
[Editor’s note: The clinical trial that Dr. Vleugels mentions can be found here.]
Tinted glass in carsParticipant:
I get a lot of sun on my arms when I drive. Would getting tinted glass on the side window help? Any particular type? Some stores sell tinted (plastic?) sheets you can peel off and stick to a window (for example if you are a passenger in someone else's car). Do those work?Dr. Ruth Ann Vleugels:
This is an option for extremely photosensitive patients, however, wearing a sun protective jacket or shirt in the care is easier and transferrable to other cars. They even make sun protective arm sleeves only to slip on in the car. (Check online.) Some states have restrictions on tinting your car glass. In some states, a doctor can write a note to get permission for the patient to have tinted glass.
Testing for remission or healingParticipant:
Hello. Thank you for answering my question. I was diagnosed ADM with anti-TIF1-gamma antibodies borderline/positive in December 2016. I took no meds except low-dose naltrexone and use gluten/soy/corn-free diet, meditation, and exercise. In past 18 months, all symptoms have diminished to the point that I only have a little pink around a few cuticles. Are there any blood tests that can help determine if I still have markers? Recent ANA was negative. Is it possible to totally heal? Have you seen it?Dr. Ruth Ann Vleugels:
Unfortunately, there is no blood test to tell you whether you are in remission in terms of skin disease. We need to do this by an expert clinical exam. A change in the autoantibody studies will not help us.
As above, patients can go into remission, but we would usually see no residual skin rash, and the patient would have no activity of the rash at their nailfolds (blood vessels near the nails) for this to be the case.
Role of auto antibodies in skin-predominant DM?Participant:
Do you use the autoantibody status of a patient to decide which drug or treatment regimen to try first? Does the regimen change with mild versus severe skin disease, or recalcitrant skin disease and autoantibody status? Or does the treatment decision have more to do with what other issues may be present (such as several muscle involvement or lung disease, for example) versus autoantibody status?Dr. Ruth Ann Vleugels:
1) Most important is whether the patient has muscle or lung disease. This will guide treatment.
2) If skin disease is the guide, we then go to disease severity. I start with much more aggressive medication in a patient with severe disease.
3) We are slowly learning which autoantibody types may respond best to which medications. This is an emerging body of knowledge, but we are not there yet for skin disease for the most part.
Allergy to prednisoneParticipant:
Hi! I have recently been diagnosed with dermatomyositis with myopathy and ILD. I also have heart involvement. I am not on treatment right now, because I am allergic to prednisone, and I am waiting on my appointment with somebody knowledgeable in myositis. What other options would you suggest?
To really mix things up, I also have mast cell activation syndrome, which makes me often react very, very strongly to medications, and I get a lot of negative side effects.Dr. Ruth Ann Vleugels:
Where do you live? Please let TMA know, so we can connect you quickly with an expert. There are many options to consider quickly, even if you are allergic to prednisone. For example, I start most patients with any ILD on CellCept right away. This takes longer to take effect, but at least can start working while you are waiting to determine whether you could get an alternative steroid. There are many other options if your disease is severe.
Have you found any evidence of flare ups being related to sun and heat exposure?Participant:
I was diagnosed with dermatomyositis by muscle biopsy several years ago. I have had only two major flare ups. Both times were in the heat of summer. (We reach 95-100 degrees.) I had been outdoors and walking in brush. Both legs and feet from the knees down became swollen. I could not wear shoes. Both legs were covered with a very dark purple rash so dense you could barely see light skin through the purple. My feet were very swollen and painful to walk on. Doctors at first considered insect bites or grass/weed allergy. None could come to any conclusion. Steroids were given, and I didn’t have other symptoms for some time.
I connected heat and sun mainly because it seemed similar to the blistered skin my dad had when he worked in the summer heat. Any exposed skin was affected. In later years he became crippled with rheumatoid arthritis. I would be very interested to know if heat and/or sun has been found to trigger flare ups like this in other autoimmune diseases as well as in myositis. Thank you.Dr. Ruth Ann Vleugels:
- Sun definitely flares dermatomyositis. It is the sun, not the heat per se.
- The rash that you describe on the lower legs does not sound like a typical DM rash. Perhaps it was leukocytoclastic vasculitis or another rash with this deep purple color. We can see this in patients with autoimmune disease or those who have started new medications, etc.
Do you have a recommendation for the chronic skin itching?Participant:
This itching is on and under the skin. There are times a skin rash will surface and itch as well. Usually no rash appears. The itching also changes location of severity as my whole body itches continuously.Dr. Ruth Ann Vleugels:
Hydrate your skin as much as possible. Don’t use very hot showers, and apply a thick emollient every day (for example Cerave cream rather than lotion). If the itch is from DM, however, we need to increase your treatment regimen to get rid of itch.
What is the effectiveness of IVIg for DM? I have tried most other meds, which did not work or had bad side effects. Thanks!Dr. Ruth Ann Vleugels:
I think this is one of the best options I have for skin disease in DM and often use it in my refractory patients. It is also considered extremely safe. If it was not expensive, the medical community would use it very early on in most patients for skin disease (in my opinion). This often is not allowed by insurance, but once you have failed some treatment options, we can usually get IVIg for our patients, and for many it works extremely well. Thankfully, there has been a randomized controlled trial for this medication in DM in the early 1990s that showed benefit.
I have many calcium deposits under the skin. They are not noticeable on the surface, but they feel like lumps under the skin. I have had biopsies, and they indicate calcium deposits. I have had tonsil cancer, so I am concerned. What do you recommend for treating calcium deposits?Dr. Ruth Ann Vleugels:
Calcinosis in DM is one of our most challenging parts of treating this disease. Here is how we think about this as I have several questions about this:
1) If you are getting new areas of calcium deposited, we have to consider whether your disease is still active and if so, treat it more aggressively. I
f this is the case, we can get improvement in small, recently laid down areas of calcium.
2) If the calcium deposits are all old and particularly if they are large, we do not currently have good treatments for this.
We try many things:
- If one or two lesions are particularly bothersome, we remove them surgically, and this works well for some patients.
- If they are extremely extensive, we try high-dose diltiazem, IV pamidronate, IVIg, etc. But usually we only get mild improvement rather than dramatic results (which is very disappointing for our patients).
- We are continually trying new treatments to see if they will help patients with this problem
- If they are over joints, do physical therapy or exercise to keep those joints mobile.
Avoiding sun exposureParticipant:
Dear Professor Vleugels. I cannot find information on whether the problem with sun exposure is a localized or a systemic effect. In practical terms, do I need to only protect my hands (where my rash is), and my face (because I could get a rash there), or is there evidence that I also need to protect, for example, my arms and legs and back? Thank you.Dr. Ruth Ann Vleugels:
I would use sun protection at the very least from the waist up. Many of my patients can get away with wearing shorts/skirts with no trouble (only for some is this the case).
Flaring of internal disease after sun exposure has been reported but is much less common than with lupus.
In skin disease only patients, we are not currently concerned that sun would create internal disease
Plaquenil and genericsParticipant:
Dr. Vleugels, when I first went on Plaquenil (generic) I experienced pretty bad diarrhea. I switched a second generic, which actually arrived as the name branded Plaquenil, and I seem to tolerate that much better. Is this issue with the source of the drug common?Dr. Ruth Ann Vleugels:
It is very common.
Generic hydroxychloroquine causes GI distress in approximately 10-20% of patients.
When we switch to brand-name Plaquenil, this usually goes away.
I bruise more easily than I should. For example, I slipped and hit my leg on the side of a jacuzzi and the bruise was huge and dark on both legs, and it stayed that way for over a month. Do certain medications cause this more?Dr. Ruth Ann Vleugels:
Yes, prednisone in particular, even in the past, can be related to this
So can aspirin and other blood thinners
There are other medications as well
I live in upstate New York. Can you recommend to TMA a dermatologist.Participant:
The dermatologists located here are not familiar with this disease and are not sure how to treat. I am considered a person with a very rare disease.Dr. Ruth Ann Vleugels:
In New York City: Dr. Alisa Femia at NYU. I hope that is an option+.
I also see many patients from upstate NY in Boston
Should a DM person have Botox for extreme headaches?Participant:
I suffer really bad headaches, and this was a suggestion.Dr. Ruth Ann Vleugels:
There is no contraindication to this treatment from a DM perspective.
We actually use Botox to the hands for refractory Raynaud’s in patients with severe Raynaud’s in the setting of autoimmune disease.
Can you discuss any natural remedies you have found that work well for patients? I’m trying to use less of the steroids and medications. Thanks!Dr. Ruth Ann Vleugels:
What I can recommend is healthy sleep, healthy diet, healthy stress reduction.
There is no evidence that cutting a particular food out will help.
Stress clearly flares autoimmune disease. We see this in practice all the time. This can be emotional stress or physical stress on the body. No one can be stress free! But find things that help you, like walking, yoga, art, etc. This is important.
This concludes today's live discussion. We'd like to extend a special thank you to Dr. Vleugels for taking the time to answer your questions, as well as all of our participants.Dr. Ruth Ann Vleugels: