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June 2026

Recognizing and Treating Cutaneous Lupus and Dermatomyositis

By ODAC Sessions

autoimmune connective tissue disorders

At the ODAC Dermatology Conference, Anthony Fernandez, MD, PhD, FAAD, shared his experience in autoimmune connective tissue disorders, highlighting the intersection between dermatology and rheumatology, and underscoring the importance of practical approaches in complex diseases.

Cutaneous Lupus

Dr. Fernandez began his session by addressing cutaneous lupus, stressing the need to recognize lesion patterns. “If you become familiar with the morphology of the skin lesions and the pattern of distribution, you should be able to recognize lupus and dermatomyositis.”

Regarding acute cutaneous lupus, he noted that, “All of these patients have systemic lupus erythematosus (SLE), so [they] are going to have a positive ANA and usually numerous positive anti-ENA antibodies.” However, he also cautioned, “Not every patient has a classic butterfly rash, so you need to look at the entire clinical picture.”

For subacute cutaneous lupus (SCLE), he emphasized photosensitivity. “Annular scaly lesions or psoriasiform lesions in a photo-distribution are a hallmark. About 25-40% of these patients may develop SCLE because of a medication they’re taking, so you need to perform a good medication history upon diagnosis.”

Chronic cutaneous lupus, particularly discoid lupus (DLE), carries unique implications. “A negative ANA does not rule out cutaneous lupus, especially with discoid lesions,” he stressed. “Patients with generalized discoid lupus, meaning they have lesions both above and below the neck, are at increased risk for progressing to SLE compared to patients with DLE lesions only above the neck.”

Dr. Fernandez advised antimalarials, hydroxychloroquine, and chloroquine as first-line systemic treatments for cutaneous lupus. “If a patient fails the first antimalarial, there is research supporting that switching to the alternative antimalarial may be effective in a significant percentage of cases.”

Dermatomyositis

Turning to dermatomyositis, Dr. Fernandez highlighted hallmark cutaneous features: heliotrope rash, Gottron’s papules, shawl sign, V-neck sign, and holster sign. He noted that any combination of these features may occur in patients with dermatomyositis, and cautioned about MDA5-positive disease. “These patients often present with skin lesions due to vasculopathy. There is a very strong risk of interstitial lung disease (ILD). Some may have a rapidly progressive ILD phenotype, which can be life-threatening despite aggressive treatment.”

For diagnosis, he recommended combining clinical and objective data. “You need to correlate what you see clinically with some objective data, mainly a lesional skin biopsy with histopathologic characteristics consistent with lupus or dermatomyositis.” He also advised autoantibody testing. “For dermatomyositis, myositis-specific autoantibodies correlate with clinical phenotype, so they have tremendous value in terms of how you test, monitor, and treat these patients.”

For treatment, Dr. Fernandez recommended typically starting with corticosteroids plus a steroid-sparing agent. “If patients fail traditional agents, IVIG is far and away the best medicine we currently have for treating the skin, myositis, or both.”

Looking ahead, he concluded with excitement about emerging therapies. “Oral JAK inhibitors, anti-interferon receptor monoclonal antibodies, and anti-interferon-beta monoclonal antibodies may be the first truly novel medicines that get approved for dermatomyositis in decades, depending upon results of ongoing phase 3 trials.” He conveyed to attendees, “Once you make a diagnosis, we currently have good medicines to offer, but the real excitement is what’s in the pipeline right now.”

This information was presented at the 2026 ODAC conference by Anthony Fernandez, MD, PhD, FAAD. The above highlights from this lecture were written and compiled by Samip Sheth, MD.