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痒疹性表皮松解症用巴瑞替尼治疗:一例报告。

Epidermolysis Bullosa Pruriginosa treated with baricitinib: A case report.

机构信息

Shanxi Medical University, Taiyuan, Shanxi, China.

Department of Dermatology, First Hospital of Shanxi Medical University, Taiyuan, Shanxi, China.

出版信息

Medicine (Baltimore). 2024 Jul 5;103(27):e38854. doi: 10.1097/MD.0000000000038854.

Abstract

INTRODUCTION

Epidermolysis Bullosa Pruriginosa (EBP) is a persistent, recurring disease that seriously affects quality of life. Fewer than 100 cases of 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.

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 has seriously affected the patient normal life.

DIAGNOSIS

The patient was diagnosed with EBP 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 skin rashes had subsided and flattened remarkable, and his itching was markedly relieved. The visual analogue scale (VAS) itching score of the patient gradually declined from 8 to 9 points to 2 to 3 points.

CONCLUSION

This study confirms that baricitinib is effective and feasible in treating EBP, especially in remarkable relieving itching, which rendered new ideas for therapeutic approaches for EBP in the future.

摘要

简介

瘙痒性大疱性表皮松解症(EBP)是一种持续性、复发性疾病,严重影响生活质量。迄今为止,报告的 EBP 病例少于 100 例。许多炎症性皮肤病是由可溶性炎症介质驱动的,这些介质依赖于 Janus 激酶信号转导和转录激活因子(JAK-STAT)信号通路,使用 Janus 激酶(JAK)抑制剂抑制该通路可能是这些疾病的一种有用的治疗策略。

患者关注

一名 28 岁男性因躯干和四肢反复出现丘疹、结节和剧烈瘙痒 12 年而到我院就诊。反复出现的严重瘙痒严重影响了患者的正常生活。

诊断

根据检查结果,患者被诊断为 EBP。

干预措施

口服巴瑞替尼片(2mg,每天一次)+口服地氯雷他定枸橼酸二钠片(8.8mg,每天一次),联合外用复方氟米松软膏和夫西地酸乳膏。

结果

患者皮疹已消退,明显变平,瘙痒明显缓解。患者的视觉模拟评分(VAS)瘙痒评分从 8 分至 9 分逐渐降至 2 分至 3 分。

结论

本研究证实巴瑞替尼治疗 EBP 有效且可行,特别是在显著缓解瘙痒方面,为未来 EBP 的治疗方法提供了新的思路。

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