Amelioration of dystrophic epidermolysis bullosa pruriginosa symptoms with dupilumab: A case report

Wang, Yangbin; Zhou, Mingming; Zhang, Li; Zheng, Song Guo; Hong, Yuxiao; Gao, Xing‐Hua

doi:10.1111/dth.15130

Cited by 20 publications

(32 citation statements)

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“…In most of the patients, skin inflammation significantly improved after drug initiation; however, the serum inflammatory cytokine level (IL‐1β, IL‐17A, IL‐1RA, IL‐18, CXCL9) showed only minor changes according to one report by Süßmuth et al 19 . Aside from the amelioration of skin lesions, an improvement in hair growth with less broken hairs was observed in three patients treated with dupilumab 17,22 . Murase et al.…”

Section: Discussionmentioning

confidence: 95%

“…After dupilumab treatment, the patients' hair grew longer, thicker and smoother without features of bamboo hair. 22 Both studies demonstrated that dupilumab treatment could potentially improve hair condition and quality of life in NS patients. Currently, a randomized doubleblind placebo-controlled trial is being conducted to further investigate the efficacy of dupilumab among these patients (trial number: NCT04244006).…”

Section: Discussionmentioning

confidence: 99%

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Dupilumab in the treatment of genodermatosis: A systematic review

Dai

et al. 2023

J Deutsche Derma Gesell

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Summary Dupilumab interferes with the signaling pathways of IL‐4 and IL‐13 and is effective in treating atopic dermatitis. Specific genodermatoses, including Netherton syndrome, epidermolysis bullosa pruriginosa, and hyper‐IgE syndrome, are Th2 skewed diseases with activation of type 2 inflammation. We performed this systematic review to investigate the therapeutic role of dupilumab in the treatment of genodermatosis. A systematic search was conducted of the PubMed, Embase, Web of Science, and Cochrane databases from inception to December 13, 2021. The review included studies with relevant terms including “dupilumab,” “genodermatosis”, “Netherton syndrome”, “ichthyosis”, “epidermolysis bullosa” and “hyper‐IgE syndrome”. The initial search yielded 2,888 results, of which 28 studies and 37 patients with genodermatosis were enrolled. The assessed genodermatoses included Netherton syndrome, epidermolysis bullosa pruriginosa, hyper‐IgE syndrome, Hailey‐Hailey disease, and severe eczema associated with genetic disorders. Most of the reported cases showed significant clinical improvement after the initiation of dupilumab treatment without major adverse events. Decreased immunoglobulin E levels and cytokine normalization have also been documented. In conclusion, Dupilumab may have a potential therapeutic role in certain genodermatoses skewed towards T helper 2 (Th2) immunity, including Netherton syndrome, epidermolysis bullosa pruriginosa, hyper‐IgE syndrome, Hailey‐Hailey disease, and severe eczema associated with some genetic disorders.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Dupilumab in the treatment of genodermatosis: A systematic review

Dai

et al. 2023

J Deutsche Derma Gesell

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“…berichteten ebenfalls über eine NS‐Patientin mit dermatoskopischem Nachweis von Bambushaaren. Nach der Dupilumab‐Behandlung wuchsen die Haare der Patientin länger, waren dicker und glatter und wiesen keine Merkmale von Bambushaar mehr auf 22 . Beide Studien zeigten, dass die Behandlung mit Dupilumab möglicherweise den Haarzustand und die Lebensqualität bei NS‐Patienten verbessern kann.…”

Section: Diskussionunclassified

Dupilumab zur Behandlung von Genodermatosen: Eine systematische Übersicht

Dai

et al. 2023

J Deutsche Derma Gesell

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Zusammenfassung Dupilumab greift in die Signalwege von IL‐4 und IL‐13 ein und wird erfolgreich zur Behandlung der atopischen Dermatitis eingesetzt. Genodermatosen wie das Netherton‐Syndrom, die Epidermolysis bullosa pruriginosa und das Hyper‐IgE‐Syndrom sind Th2‐vermittelte Erkrankungen mit Aktivierung einer Typ‐2‐Entzündung. In dieser systematischen Übersicht haben wir die therapeutische Rolle von Dupilumab bei der Behandlung von Genodermatosen untersucht. Dazu wurden die Datenbanken PubMed, Embase, Web of Science und Cochrane seit ihrer Einführung bis zum 13. Dezember 2021 systematisch durchsucht. Die Übersicht umfasst Studien mit relevanten Begriffen, einschließlich „Dupilumab“, „Genodermatose“, „Netherton‐Syndrom“, „Ichthyose“, „Epidermolysis bullosa“ und „Hyper‐IgE‐Syndrom“. Die anfängliche Suche ergab 2888 Treffer; davon wurden 28 Studien und 37 Patienten mit Genodermatosen eingeschlossen. Zu den beurteilten Genodermatosen gehörten Netherton‐Syndrom, Epidermolysis bullosa pruriginosa, Hyper‐IgE‐Syndrom, Morbus Hailey‐Hailey und schwere Ekzeme im Zusammenhang mit genetischen Erkrankungen. Die meisten der beschriebenen Fälle zeigten eine signifikante klinische Verbesserung nach Einleitung der Dupilumab‐Behandlung ohne wesentliche unerwünschte Ereignisse. Zudem wurden eine Verringerung der Immunglobulin‐E‐Spiegel und eine Normalisierung der Zytokinspiegel dokumentiert. Zusammenfassend hat Dupilumab möglicherweise eine therapeutische Rolle bei bestimmten Genodermatosen mit einer abnormal gesteigerten Typ‐2‐T‐Helfer‐Immunität (Th2), einschließlich Netherton‐Syndrom, Epidermolysis bullosa pruriginosa, Hyper‐IgE‐Syndrom, Morbus Hailey‐Hailey und schweren Ekzemen bei genetischen Erkrankungen.

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“…4,49,50 Recently, six previous reports have documented successful treatment of DEB-Pr with dupilumab, a monoclonal antibody targeting the IL-4 receptor alpha (IL-4Rα) that provides dual blockade of IL-4 and IL-13, which play an important role in the pathogenesis of AD. 4,26,[51][52][53] In addition, Darbord et al 54 reported mast cell infiltration of the lesional skin, high serum total IgE levels and increased Th2 subsets in circulating T cells in patients with DEB-Pr. These findings suggest that DEB-Pr is driven by the Th2 immune response.…”

Section: Ta B L E 2 (Continued)mentioning

confidence: 99%

New insight of itch mediators and proinflammatory cytokines in epidermolysis bullosa

Nguyen

Shinkuma

Katsumi

et al. 2022

J Cutaneous Imm & Allergy

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Objectives Epidermolysis bullosa (EB) is a hereditary disorder characterized by mechanical stress‐induced blistering. The presence of extracutaneous complications such as cardiomyopathy and renal disease observed in severe EB subtypes and the fact that pruritus is a common symptom across all EB subtypes indicate that EB is not only a skin fragility disease but also a systemic inflammatory disorder. Our study aims to elucidate the basis of the systemic inflammation seen in EB patients. Methods We analyzed serum samples of 20 EB patients by Luminex bead‐based cytokine assays and enzyme‐linked immunosorbent assays. Results The serum levels of sIL‐2R, IL‐6, HGF, M‐CSF, SCGF‐β, IL‐8, IL‐16, IFN‐γ, MIF, MIP‐1α, and thymic stromal lymphopoietin (TSLP) (p < .01, p < .01, p < .01, p < .01, p < .01, p < .05, p < .05, p < .05, p < .05, p < .05, and p = .01, respectively) were found to be significantly elevated in EB patients, whereas TNF‐β (p < .01) was decreased. Th2 cytokines including IL‐4, IL‐5, and IL‐13 were not elevated in EB patients. In contrast, TSLP was significantly increased, and IL‐31 and oncostatin M were not statistically significant but tended to be higher in the patients than in healthy controls. Among proinflammatory cytokines such as IL‐1β, IL‐6, and TNF‐α, IL‐6 was elevated in EB patients. Conclusions The imbalance of several itch mediators and proinflammatory cytokines was identified. Biologics targeting the cytokines found to be elevated in the sera of patients is considered as a beneficial treatment option for EB.

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Amelioration of dystrophic epidermolysis bullosa pruriginosa symptoms with dupilumab: A case report

Cited by 20 publications

References 4 publications

Dupilumab in the treatment of genodermatosis: A systematic review

Dupilumab in the treatment of genodermatosis: A systematic review

Dupilumab zur Behandlung von Genodermatosen: Eine systematische Übersicht

New insight of itch mediators and proinflammatory cytokines in epidermolysis bullosa

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