Treatment of epidermolysis bullosa pruriginosa‐associated pruritus with dupilumab

Shehadeh, Waseem; Sarig, Ofer; J, Bar; Sprecher, Eli; Samuelov, Liat

doi:10.1111/bjd.18855

Cited by 55 publications

(69 citation statements)

References 8 publications

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“…One other report of dupilumab in EBP demonstrated improvement after 12 weeks. 1 Our patients demonstrate sustained improvement, and in Patient 2, resolution of skin findings after increased dosing. The mechanism of dupilumab in EBP is unclear but may involve blocking IL-4Rα-mediated sensitization of sensory neurons to pruritogens.…”

Section: Pediatric Dermatologymentioning

confidence: 54%

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Epidermolysis bullosa pruriginosa treated with dupilumab

Zhou

Little

Antaya

2020

Pediatric Dermatology

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Epidermolysis bullosa pruriginosa (EBP) is a variant of dystrophic epidermolysis bullosa characterized by intense pruritus and prurigo nodularis-like lesions. While medical therapies for EBP exist, current treatments are not consistently effective, and symptoms often cause decreased quality of life. Here, we report two cases of EBP treated with dupilumab, which decreased symptoms of pruritus and improved skin findings. Both patients have been on dupilumab for over one year with sustained improvement and no adverse effects; although in one patient, increased dosing was required to attain optimal control of disease.Epidermolysis bullosa pruriginosa (EBP) is a variant of dystrophic epidermolysis bullosa caused by COL7A1 mutations. 1 Current treatments are not consistently effective. 2 Dupilumab, a monoclonal antibody against IL-4 receptor alpha (IL4-Rα), is approved for atopic dermatitis (AD) and has been used for prurigo nodularis (PN), 3 which shares clinical features with EBP. We report successful treatment of EBP with dupilumab.

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Section: Pediatric Dermatologymentioning

confidence: 54%

“…Little is supported by a Career Development Award from the Dermatology Foundation. Amanda G. Zhou BS 1…”

Section: Ack N Owled G M Entsmentioning

confidence: 99%

Epidermolysis bullosa pruriginosa treated with dupilumab

Zhou

Little

Antaya

2020

Pediatric Dermatology

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“…[31] Emerging and ongoing clinical trials and therapeutic approaches for Epidermolysis dupilumab, a monoclonal antibody approved for atopic dermatitis that targets IL-4Rα, showed clinical efficacy in a 52-year-old patient with a highly pruriginous subtype of EB (EB pruriginosa). [33] Dupilumab led to a rapid and significant clinical improvement of disabling itch and prurigo-like skin lesions, although its impact on pruritogenic and inflammatory pathways in EB remains to be determined. [33][34][35] Chronic pruritus constitutes a major individual complaint in many EB patients, often inducing a vicious itch-scratchblister cycle that is frequently inadequately controlled and resistant to treatments.…”

Section: Symp Tom -Relie Ving and Dis E A S E-modif Ying Ther Apie Smentioning

confidence: 99%

“…[ 33 ] Dupilumab led to a rapid and significant clinical improvement of disabling itch and prurigo‐like skin lesions, although its impact on pruritogenic and inflammatory pathways in EB remains to be determined. [ 33–35 ] Chronic pruritus constitutes a major individual complaint in many EB patients, often inducing a vicious itch‐scratch‐blister cycle that is frequently inadequately controlled and resistant to treatments. [ 36 ] Its intensity correlates with the severity of the EB subtype.…”

Section: Symptom‐relieving and Disease‐modifying Therapiesmentioning

confidence: 99%

Translational perspectives to treat Epidermolysis bullosa—Where do we stand?

Prodinger

Bauer

Laimer

2020

Experimental Dermatology

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Epidermolysis bullosa (EB) comprises a group of rare, genetically determined skin fragility disorders characterized by (muco-) cutaneous blistering following mild mechanical trauma. The most recent classification guidelines published in February 2020 define EB as the prototypic genetic disorder of skin fragility. Other skin fragility disorders, including peeling skin disorders, erosive disorders, hyperkeratotic disorders and connective tissue disorders with skin fragility, are now classified as a separate category, that is so-called

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“…If the itch associated with EB is in fact a mechanism of inflammatory mediators as part of the skin healing process, a promising area for investigation is the role of the Th‐2 immune axis and its cytokines IL‐4 and IL‐13, which have been shown to play a key role in fibrosis and wound healing 9 . In fact, at least one case report shows a patient's rapid and significant improvement after treatment with dupilumab, a monoclonal antibody that selectively blocks this cytokine pathway 10 . Another area of study is the substance P‐neurokinin 1 receptor (NK1R) pathway that is implicated in many chronic itch conditions.…”

Section: Epidermolysis Bullosamentioning

confidence: 99%

Genodermatoses with itch as a prominent feature

Fourzali

Yosipovitch

2020

Acad Dermatol Venereol

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A number of inherited conditions cause chronic itch as a part of the recognized phenotype. Advances in the understanding of the genetic factors that cause these diseases elucidate the molecular underpinning of itch as a symptom. Our knowledge of the causes of chronic itch has also advanced, providing an opportunity to integrate the genetic pathophysiology with the molecular landscape of chronic itch mediators. This article reviews select genodermatoses that have itch as a predominant feature with a focus on the pathophysiology of the disease, how it may lead to itch and potential therapeutic targets.

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Treatment of epidermolysis bullosa pruriginosa‐associated pruritus with dupilumab

Cited by 55 publications

References 8 publications

Epidermolysis bullosa pruriginosa treated with dupilumab

Epidermolysis bullosa pruriginosa treated with dupilumab

Translational perspectives to treat Epidermolysis bullosa—Where do we stand?

Genodermatoses with itch as a prominent feature

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