Dermatologist offers advice for treating 'horribly refractory' dermatomyositis
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A number of approaches and therapies may be necessary to treat cutaneous dermatomyositis, according to a presenter at the 2021 Congress of Clinical Rheumatology-West.
“Dermatomyositis, as a dermatologist, is one of the hardest skin diseases we treat,” Ruth Ann Vleugels, MD, MPH, vice chair for academic affairs in the department of dermatology at Harvard Medical School. “It is horribly refractory.”
Vleugels framed the discussion in terms of a “therapeutic ladder.” Following a stepwise approach will ensure that all aspects of cutaneous dermatomyositis are addressed, she said.
The first message was simple. Photoprotection is critical in these patients and should involve sunscreen and protective clothing. Vitamin D supplementation may be considered, as well.
The next step was also straightforward. “Don’t forget to treat itch,” Vleugels said, and reminded attendees that this is an inflammatory disease. “If your patient is itchy, it means their disease is not adequately controlled.”
The next rung on the ladder included topical approaches, including corticosteroids or tacrolimus. However, Vleugels reminded the audience that topicals are “essentially adjunctive” to systemic therapies in most patients. “Of my over 500 patients, just a couple are only on topical therapies, and they have very minor, very mild disease,” she said.
Regarding systemic therapies, while some clinicians may be tempted to reach for hydroxychloroquine, Vleugels said this drug can be associated with a rash in about one-third of patients. “I still consider it because of the photoprotective benefit,” she said. “But I only use it for mild, mild disease.”
Overall, antimalarials are likely to only be sufficient monotherapy in about 10% of patients, according to Vleugels. It is for this reason that she suggested they should be used in combination with other treatments on the therapeutic ladder. “If a patient comes into my clinic with moderate or severe skin disease, on day 1, I am not going to start them on antimalarials alone,” she said.
For those moderate or severe patients, the “vast majority” will begin with methotrexate or mycophenolate mofetil (MMF), according to Vleugels. Regarding the choice between the two in the first line, Vleugels offered two reasons why methotrexate may be attractive.
One concerns cancer patients with dermatomyositis. “Oncologists do not care about methotrexate,” she said. “It is a great agent that does not immunosuppress the patient more.”
The other reason to choose methotrexate is that it is an effective steroid-sparing agent with an acceptable safety profile.
Conversely, there is one patient group for whom MMF is the better first line option: those with lung involvement.
Moving beyond these two drugs, Vleugels said that intravenous immunoglobulin is often the next rung on the ladder — and highly effective — for those “horribly refractory” patients.
In fact, Vleugels believes that this drug may be the future of treatment in this disease. “If it were inexpensive and easy to give, I would give IVIG to every cutaneous dermatomyositis patient on the first day,” she said. “That is how confident I am that it works in the vast majority of patients. If you are not using it in your refractory skin patients, you are missing the ultimate therapy we have.”
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Vleugels RA. Dermatology for the Rheumatologist. Presented at: Congress of Clinical Rheumatology-West annual symposium; September 18-21, 2021 (hybrid meeting).
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