Epidermolysis Bullosa Pruriginosa Treated with Baricitinib:A
Case
Report
Zhe He1, PhD
Qian Dong1, PhD
Yue Xi 1, PhD
Rui Zheng2, PhD
1 Shanxi
Medical University, Taiyuan, Shanxi, China; 2Department of Dermatology, First Hospital of Shanxi Medical University,
Taiyuan, Shanxi, China.
Correspondence: Rui Zheng, Email:Zhengr2002@163.com
EBP: Epidermolysis Bullosa Pruriginosa
JAK-STAT: Janus kinase-signal transducer and activator of transcription
JAK: Janus kinase
DEB: Dystrophic Epidermolysis Bullosa
VAS: The Visual Analogue Scal
Abstract : 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