Zhe He

and 3 more

Epidermolysis Bullosa Pruriginosa Treated with Baricitinib:A Case ReportZhe He1, PhDQian Dong1, PhDYue Xi 1, PhDRui Zheng2, PhD1 Shanxi Medical University, Taiyuan, Shanxi, China; 2Department of Dermatology, First Hospital of Shanxi Medical University, Taiyuan, Shanxi, China.Correspondence: Rui Zheng, Email:[email protected]: Epidermolysis Bullosa PruriginosaJAK-STAT: Janus kinase-signal transducer and activator of transcriptionJAK: Janus kinaseDEB: Dystrophic Epidermolysis BullosaVAS: The Visual Analogue ScalAbstract : Introduction: Fewer than 100 cases of Epidermolysis Bullosa Pruriginosa(EBP) have been reported to date. Numerous inflammatory dermatoses are driven by soluble inflammatory mediators, which rely on Janus kinase-signal transducer and activator of transcription (JAK-STAT) signaling, and inhibition of this pathway using Janus kinase (JAK) inhibitors might be a useful therapeutic strategy for these diseases. Chronic inflammation is a hallmark of Dystrophic Epidermolysis Bullosa (DEB), thus upregulation of inflammatory cytokines and JAK signaling may play a role in DEB-related pruritus. EBP is a persistent, recurring disease that seriously affects quality of life. Patient concerns: A male patient, 28 years of age, was admitted to our hospital because of recurrent papules, nodules, and intense itching on the trunk and extremities for 12 years. Repeated large and intense itching for 12 years has seriously affected the patient’s normal life.Diagnosis: The patient was diagnosed with Epidermolysis Bullosa Pruriginosa based on examination results. interventions: Oral baricitinib tablets (2 mg, once a day) + Oral desloratadine citrate disodium tablets (8.8 mg, once a day) combined with topical compound flumethasone ointment and Fucidin cream. outcomes: The patient’s skin rashes had subsided and flattened significantly, and his itching was markedly relieved. The Visual Analogue Scal (VAS) itching score of the patient had gradually declined from 8–9 points to 2–3 points. Conclusion : this study confirms that baricitinib is effective and feasible in treating EBP, especially in significantly relieving itching, which rendered new ideas for therapeutic approaches for EBP in the future.Keywords: Epidermolysis Bullosa, kinase inhibitors, Baricitinib Treatment