The following is a summary of “Efficacy of oral JAK1 or JAK1/2 inhibitor for treating refractory pruritus in dystrophic epidermolysis bullosa: A retrospective case series,” published in the March 2024 issue of Dermatology by Kwon, et al.

Pruritus is a significant concern for patients with dystrophic epidermolysis bullosa (DEB), leading to considerable distress and contributing to an itch-scratch-blister cycle. Chronic inflammation, a characteristic feature of DEB, suggests a potential role for upregulated inflammatory cytokines and Janus kinase (JAK) signaling in the pathogenesis of pruritus. For a study, researchers conducted a retrospective review to evaluate the efficacy of two JAK inhibitors, baricitinib, and upadacitinib, in treating refractory pruritus in DEB patients.

They analyzed the medical records of DEB patients with refractory pruritus who received either baricitinib (4 mg) or upadacitinib (15 mg) once daily for a duration ranging from 2 to 32 weeks. The study cohort comprised 12 DEB patients, including six with recessive DEB and six with dominant DEB. The baseline pruritus visual analog scale (VAS) score was 7.5 ± 1.7. Changes in pruritus VAS scores and other relevant parameters were assessed.

Treatment with either upadacitinib or baricitinib resulted in rapid and sustained reductions in pruritus. At weeks 2 and 4, 33.3% and 70% of patients demonstrated a decrease of at least 3 points in the pruritus VAS score from baseline. The mean percentage changes from baseline in pruritus VAS scores at weeks 2 and 4 were −42.9% and −52.7%, respectively. Subgroup analysis (n = 5) revealed potentially more significant reductions in the pruritus VAS score with baricitinib compared to upadacitinib and in patients with epidermolysis bullosa pruriginosa (n = 3) compared to other DEB subtypes. Reductions in pain intensity and the number of new blisters were observed and correlated with reductions in the pruritus score. No serious adverse events necessitated treatment discontinuation.

JAK1 or JAK1/2 inhibitors showed promise as therapeutic options for DEB-related pruritus, with sustained reductions in pruritus severity. Long-term safety assessments were warranted to evaluate their clinical utility further.

Reference: onlinelibrary.wiley.com/doi/10.1111/1346-8138.17079