Epidermolysis bullosa pruriginosa with extensive truncal involvement treated with upadacitinib

Kim, Na Young; Jue, Mihn‐Sook; Huh, Yun Jung; Sung, Kyung Jeh; Kim, Hyunsung; Kim, Jeong Eun; Ko, Joo Yeon

doi:10.1111/jdv.18487

Cited by 8 publications

(7 citation statements)

References 6 publications

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“…Previously, JAK inhibitors demonstrated success in treating itching in patients with EBP 17,18 . Our study included three EBP patients out of the 12 DEB patients.…”

Section: Discussionmentioning

confidence: 99%

“…Recently, two cases of EB pruriginosa (EBP), an unusual subtype of DEB characterized by intensely pruritic prurigo-like lesions with the typical DEB features, were reported to be successfully treated with a JAK1/2 inhibitor, baricitinib, and a JAK1-selective inhibitor, upadacitinib. 17,18 However, limited data exist on the use of JAK inhibitors in other DEB subtypes. This study aimed to evaluate the potential effectiveness of a JAK1/2 inhibitor and a selective JAK1 inhibitor in relieving the refractory pruritus associated with various subtypes of DEB.…”

Section: Introductionmentioning

confidence: 99%

“…Recently, two cases of EB pruriginosa (EBP), an unusual subtype of DEB characterized by intensely pruritic prurigo‐like lesions with the typical DEB features, were reported to be successfully treated with a JAK1/2 inhibitor, baricitinib, and a JAK1‐selective inhibitor, upadacitinib 17,18 . However, limited data exist on the use of JAK inhibitors in other DEB subtypes.…”

Section: Introductionmentioning

confidence: 99%

“…17,18 Our study included three EBP patients out of the 12 DEB patients. Subgroup analysis revealed that patients with EBP presented with a higher baseline pruritus VAS score compared to other subtypes (8.7 ± 1.2 vs. 7.1 ± 1.8, p = 0.19), although not statistically significant.…”

mentioning

confidence: 99%

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Efficacy of oral JAK1 or JAK1/2 inhibitor for treating refractory pruritus in dystrophic epidermolysis bullosa: A retrospective case series

Kwon,

Kim,

Kim

et al. 2023

The Journal of Dermatology

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Refractory pruritus is the most distressing, disease‐related symptom in patients with dystrophic epidermolysis bullosa (DEB), inducing an itch‐scratch‐blister cycle. Chronic inflammation is a hallmark of DEB, thus upregulation of inflammatory cytokines and Janus kinase (JAK) signaling may play a role in DEB‐related pruritus. We retrospectively reviewed the medical records of DEB patients with refractory pruritus who were treated with either baricitinib, a JAK1/2 inhibitor, or upadacitinib, a selective JAK1 inhibitor. Patients received baricitinib (4 mg) or upadacitinib (15 mg) once a day for 2–32 weeks. A total of 12 DEB patients (six recessive DEB and six dominant DEB) were included in this study. The mean±SD baseline pruritus visual analog scale (VAS) score was 7.5 ± 1.7. Upadacitinib or baricitinib treatment resulted in a rapid and sustained decrease in itch. Four out of 12 patients (33.3%) and seven out of 10 patients (70%) showed a decrease of at least 3 points in the pruritus VAS score from baseline at weeks 2 and 4, respectively. The mean percentage changes from baseline in pruritus VAS scores at weeks 2 and 4 were −42.9% and −52.7%, respectively. Subgroup analysis showed greater reductions in the pruritus VAS score in the baricitinib group (n = 5) compared to the upadacitinib group (n = 7), and in patients with epidermolysis bullosa pruriginosa (n = 3) compared to other subtypes of DEB (n = 9); however, these differences did not reach statistical significance. Three out of 10 (33.3%) patients showed at least a 2‐point reduction in pain intensity from baseline at week 4. Eight out of 12 patients (66.7%) also showed a reduction in the number of new blisters, which correlated with a reduction in the pruritus score. No patient discontinued treatment because of serious adverse events. Our results suggest that JAK1 or JAK1/2 inhibitors could be a promising treatment option for DEB‐related pruritus. Long‐term safety should be assessed in future studies.

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“…Previously, JAK inhibitors demonstrated success in treating itching in patients with EBP 17,18 . Our study included three EBP patients out of the 12 DEB patients.…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

mentioning

confidence: 99%

See 2 more Smart Citations

Efficacy of oral JAK1 or JAK1/2 inhibitor for treating refractory pruritus in dystrophic epidermolysis bullosa: A retrospective case series

Kwon,

Kim,

Kim

et al. 2023

The Journal of Dermatology

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“…Upadacitinib has been shown to profoundly ameliorate itch in patients with pruriginous conditions like atopic dermatitis 8 but also in heritable disorders like epidermolysis bullosa. 9 This can be explained by the effects of upadacitinib on inflammatory pathways and on the neurosensory system, and more specifically on the interruption of IL-31 signalling, which is a central mediator in T-cell-mediated itch. 4 JAK1i have a direct effect on T-cells, but also on the neurosensory system by blocking IL-31 and IL-4 signalling in primary afferent sensory neurons, which carry receptors for both cytokines.…”

Section: Upadacitinib For Treatment-resistant Lichen Amyloidosismentioning

confidence: 99%