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. 2024 Aug 19;4(5):e445.
doi: 10.1002/ski2.445. eCollection 2024 Oct.

Real-world experience of using dupilumab and JAK inhibitors to manage pruritus in epidermolysis bullosa pruriginosa

Ping-Chen Hou  1 , Wilson Aala Jr  2 , Wei-Ting Tu  1 , John A McGrath  1   3 , Chao-Kai Hsu  1   2   4
Affiliations

Real-world experience of using dupilumab and JAK inhibitors to manage pruritus in epidermolysis bullosa pruriginosa

Ping-Chen Hou et al. Skin Health Dis. .

Abstract

Epidermolysis bullosa pruriginosa (EBP) is a form of dystrophic EB associated with severe pruritus and has skewed Th2 inflammation. Our study suggests that JAK inhibitors may offer superior efficacy compared to dupilumab in treating EBP. Moreover, JAK inhibitors downregulate JAK-STAT signalling and Th1/2 cell differentiation in lesional skin while not in peripheral blood.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Clinical responses and transcriptomic changes after JAKi in EB pruriginosa. (a) Less erythema and fewer excoriations after 9 months' dupilumab treatment in Patient 1. (b) Reduction and flattening of prurigo‐like lesions after 9 months' upadacitinib treatment in Patient 7. (c) Itch numeric rating score (NRS) in 5 EBP patients treated with dupilumab. (d) Itch NRS in 5 EBP patients treated with JAK1 inhibitors. (e) Principal component analysis reveals that the transcriptomic profiles of JAK inhibitor‐treated skin samples are distinct from pre‐treatment plots (and unrelated, non‐atopic controls). (f) Top 15 enriched pathways in the EBP lesional skin as compared to skin after systemic JAKi treatment.
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References

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