Abstract
INTRODUCTION: Darier's disease (DD) is a rare autosomal dominant genodermatosis associated with high morbidity and currently has no cure. Janus kinase (JAK) inhibitors are increasingly being investigated in dermatology for refractory and difficult-to-treat conditions. In this case report, we present a patient with refractory DD who showed a satisfactory response to this therapy. CASE PRESENTATION: A 42-year-old woman who developed pruritic brown papular lesions on her trunk, particularly under the breasts, groin, and neck, was presented. A biopsy confirmed DD, and she was treated with adalimumab, which failed to provide clinical improvement. The patient was then started on tofacitinib 10 mg daily, leading to disease stabilization. Due to persistent itching and new lesions, the dose was increased to 15 mg daily, resulting in significant improvement. After a few months, with most lesions resolved, the dose was reduced to 10 mg daily, successfully maintaining disease control. CONCLUSION: This report highlights the potential role of JAK inhibitors as an effective treatment option for refractory DD.