Bimekizumab as an Effective Treatment in Darier Disease: Clinical Outcomes of a Single-Center Retrospective Analysis of Patients Treated with Biologics

Abstract

Darier disease (DD) is a rare autosomal dominant genodermatosis with limited systemic therapeutic options. Retinoids are the gold standard therapy, but long-term application is frequently limited by adverse events. Case reports suggest that biologicals approved for psoriasis vulgaris or atopic dermatitis may be beneficial in DD. In this retrospective study, patients with DD who presented to the Department of Dermatology and Venerology, University Hospital Freiburg, between 2019 and 2024 were identified, and clinical efficacy, patient-reported outcomes, and histological features were analyzed in the patients treated with biologic agents in routine care. In total, 15 patients (female: 11, male: 4) with a mean age of 48.20 ± 9.16 years were treated with biologic agents. The three patients who received the type 2 inhibitors dupilumab or lebrikizumab worsened or remained unchanged and discontinued therapy by week 12. Patients treated with the interleukin-17A/F inhibitor bimekizumab showed reduction of the Investigator Global Assessment score and body surface area from a mean of 3.58 ± 0.56 at baseline to 1.80 ± 0.82 at week 24 ( p < 0.001) and from 32.00 ± 19.17% to 13.60 ± 9.05% (week 24; p < 0.001), respectively. The Dermatology Life Quality Index score decreased from 18.45 ± 9.87 to 4.78 ± 6.12 (week 24; p < 0.001). Additionally, on histopathology, the total numbers of dyskeratoses showed a trend towards reduction (31.17 ± 10.19 at baseline to 13.67 ± 13.25 at week 12; p = 0.062) with a decrease in the number of grains (10.50 ± 6.77 to 3.33 ± 3.88; p = 0.031). In summary, these real-world data indicate that bimekizumab may be a beneficial and relatively safe treatment option in DD.