Long-term use of systemic retinoids effective for Darier disease

Prolonged use of systemic retinoids was safe and effective for the treatment of patients with moderate to severe Darier disease, according to study results presented at the American Academy of Dermatology virtual meeting.

“Darier disease is a rare autosomal dominant genodermatosis with high penetrance and variable expressivity, caused by mutations in the ATP2A2,” Marina L. Vieira, MD, of the department of dermatology at Hospital das Clínicas of the University of São Paulo Medical School, and colleagues wrote. “Studies with systemic retinoids show efficacy in disseminated Darier disease, but there are only a few studies with long-term use and follow-up.”

Thirty-two patients diagnosed with Darier disease, evaluated from October 2007 to October 2017, were included in the retrospective study; 18 patients were treated with systemic retinoids.

Dosage ranged from 0.1 mg/kg to 0.5 mg/kg per day; 14 patients received acitretin for a mean of 78.7 months, seven patients received isotretinoin for a mean of 64.4 months, and four patients received etretinate for a mean of 51 months. Some patients received more than one treatment at different times.

Three patients discontinued medication use, but no patients discontinued due to adverse effects or therapeutic failure.

All patients treated with systemic retinoids had clinical improvement. Photographic records of 10 patients showed significant improvement with lesion clearing of more than 80% in five patients and moderate improvement with lesion clearing between 50% and 80% in five patients.

“In our dermatology service, we have 28 years of positive experience with systemic retinoids in Darier disease,” the authors said. “Prolonged use of systemic retinoids in low doses seems to be safe and effective for patients with moderate to severe Darier disease.”