Misha Rosenbach, MD
Without an FDA-approved medicine for sarcoidosis, physicians have looked to off-label use of pharmaceuticals to help their patients. This uncommon multisystem granulomatous disease of unknown cause affects the skin in 30 percent of patients.
Misha Rosenbach, MD, assistant professor of dermatology and internal medicine, director of the inpatient consult service, and director of the cutaneous sarcoidosis clinic at the University of Pennsylvania, Philadelphia, described how to recognize, evaluate, and treat these challenging patients during a Focus session Saturday.
Sarcoidosis occurs in all races, sexes, and ages, but it mainly affects adults under age 40. The annual incidence is 35.5 and 10.9 per 100,000 blacks and whites, respectively. The lifetime risk for blacks is 2.4 percent and for whites 0.85 percent.
“No one really understands exactly what sets off sarcoidosis, but it is suspected that genetic predisposition coupled with environmental antigen exposure leads to the inflammation,” Dr. Rosenbach said.
Environment plays a role, with exposure to insecticides, pesticides, mold, and mildew associated with mild increased risk. There also may be an occupational cause with an increased incidence in firefighters, those in the U.S. Navy, certain mechanics, and 9/11 first responders.
“It’s probably true that there are different environmental triggers that set off their granulomas inflammation and lead to genetically susceptible hosts,” said Dr. Rosenbach, adding that microbial agents are among other agents considered to be possible causes.
He shared photos and treatment advice for several types of sarcoidosis.
“If you see someone with a tattoo, and a biopsy shows granulomas inflammation, there are a few different pathways you should go down,” he said. “With a new tattoo, if they have pustules and granulomas inflammation, that’s almost always a microbacterial infection.”
In a photo series of people whose noses had varying presentations, Dr. Rosenbach said, “Look at how different all of these patients lesions look. These are just sarcoid lesions on the nose. There may actually be different clinical phenotypes that we as dermatologists can help identify and assess.”
Once a patient has been diagnosed, physicians must determine which systems are affected.
“The main thing is that 90 percent of patients have lung involvement, which is usually a nonproductive dry cough that goes on for a very long time,” Dr. Rosenbach said. “Eye involvement can be clinically asymptomatic, so everyone needs to see an ophthalmologist at baseline and every year.
“Hepatic involvement is very rarely clinically relevant, but it’s important to think about when you’re choosing medications that patients may have a sarcoid in their livers. Neurosarcoid is important, and it is almost always clinically symptomatic.”
Patients also can have a clinically asymptomatic cardiac sarcoid.
“Everyone should get an EKG, and if they have palpations they should probably get extended testing. The most common presenting sign of cardiac sarcoid is sudden death. That’s the worst presenting sign of any disease ever. So when you see someone with sarcoidosis, that’s your chance to intervene before the sudden death. All of your patients have to be screened,” Dr. Rosenbach said.
Once the diagnosis, biopsies, tests, workups, and evaluations are completed, Dr. Rosenbach ramps up treatment via a stepwise approach, starting with some degree of topical therapy for all patients.
The next line for either refactory or widespread skin disease is antimalarials, but also tetracycline-type antibiotics. For patients who don’t respond or have more extensive skin disease, methotrexate is very effective, he said. Biologics are next, with data suggesting that TNF inhibitors — particularly adalimumab and infliximab — are efficacious in the treatment of recalcitrant or widespread skin sarcoids.
“For any disease, we should balance how bad the disease is with how terrible the treatment is in terms of risks versus benefits. You should tailor your treatment toward your patients,” Dr. Rosenbach said.