Many features affect diagnosis, treatment of lupus

Christina Lam, MD

Christina Lam, MD

Cutaneous lupus erythematosus (CLE) can present as inflammatory skin disorders in several ways, and Christina Lam, MD, provided an overview and treatment approaches for common CLE manifestations March 5 during “Medical Dermatology Potpourri: Challenges Faced and Lessons Learned” (U042).

Skin disease seen with lupus can be classified into lupus-specific and lupus non-specific lesions. Discoid lupus erythematosus fits into the category of lupus-specific lesions, said Dr. Lam, assistant professor of dermatology at Boston University School of Medicine.

Although there is not a lot of data about the epidemiology of cutaneous lupus, she noted that the incidence is rare, with about four cases per 100,000 individuals. It is predominant in females.

“The risk of developing systemic lupus in patients with cutaneous lupus increases over time and seems to be higher in women than in men. Data show that about 10 percent to 12 percent of patients with cutaneous lupus go on to progress to systemic disease, with an average time to progression of about eight years,” Dr. Lam said. “This tells us it’s important to follow up with patients long-term because it can take a while for systemic symptoms to develop.”

She added that the majority of patients with cutaneous lupus who go on to develop systemic disease will have mild systemic disease and not severe disease. Smoking is associated with more severe disease in both systemic and cutaneous disease.

A distinctly photosensitive subset of cutaneous lupus is subacute cutaneous lupus that heals without scarring as opposed to discoid lupus that heals with scarring. Up to 50 percent of patients will have associated internal disease. Looking at classification of LE-associated skin disease, subacute cutaneous lupus fits into the lupus-specific lesions.

She cautioned that a variant, drug-induced subacute cutaneous lupus, has been gaining attention because it presents clinically, histopathologically, and immunologically similarly to idiopathic SCLE. The most common drug culprits have been found to be terbinafine, TNF-a inhibitors, antiepileptics, PPIs, thrombocyte inhibitors, ACE inhibitors, and NSAIDs.

“The treatment for drug-induced lupus is to withdraw the offending drug, and in general the lesions will resolve within about one to three months,” Dr. Lam said.

With so many case reports and open-label studies, dermatologists are left wondering which treatment direction to take for cutaneous LE. Dr. Lam said she generally starts with good photoprotection, behavioral modification, and smoking cessation, and then enlists topical agents. If topical corticosteroids or calcineurin inhibitors alone don’t work, she employs an antimalarial agent combined with a topical steroid due to delayed efficacy of antimalarials. She advises screening patients yearly for retinopathy to recognize toxicity associated with antimalarials. If a patient does not get relief with topicals and antimalarials, the next steps are immunosuppressive agents. Dr. Lam tries additional agents, such as retinoids, IVIg, or azathioprine, and then biologic therapies.

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