A case of dystrophic epidermolysis bullosa pruriginosa treated with dupilumab

Caroppo, Francesca; Milan, Elisa; Giulioni, Erika; Fortina, Anna Belloni

doi:10.1111/jdv.17887

Cited by 18 publications

(23 citation statements)

References 8 publications

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“…Дупилумаб показал высокую эффективность в лечении генерализованного кожного зуда при атопическом дерматите и узловатой почесухе [14]. В недавних публикациях был продемонстрирован успешный опыт применения дупилумаба у пациентов с резистентным к проводимой терапии кожным зудом при пруригинозной форме ДБЭ [13][14][15]22], что, возможно, свидетельствует о важной роли Th2-иммунного ответа в патогенезе заболевания [14], в связи с чем представленной в статье пациентке была рекомендована инициация терапии данным препаратом.…”

Section: Discussionunclassified

Pruriginosa Pattern of Dystrophic Epidermolisys Bullosa: Clinical Case

Леонова

Мурашкин

2023

Pediatr. farmakol.

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Background. Epidermolisys bullosa pruriginosa is a rare pattern of dystrophic epidermolisys bullosa and characterized by severe itching that accompanies the formation of papules, plaques and nodes primarily on the lower limbs skin and imitating prurigo nodularis. Nowadays, less than 100 cases of this disease are reported in the world, thus, the presentation of this clinical case is relevant. Clinical case description. The authors describe the clinical case of pruriginous pattern of dominant dystrophic bullous epidermolysis in 14-year-old female patient. Conclusion. Diagnosis of this disease pattern is extremely difficult and currently all treatment is limited to the symptomatic therapy in order to stop itching and prevent scarring.

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Section: Discussionunclassified

Pruriginosa Pattern of Dystrophic Epidermolisys Bullosa: Clinical Case

Леонова

Мурашкин

2023

Pediatr. farmakol.

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“…20,25 In EB, the itch is a common symptom with a significant impact on quality of life and is often accompanied by pain and skin infections in the patients. 10,11,26 In a study of the prevalence of itch in 40 adults with either of three EB types (EBS, JEB, or DEB), 85% reported itch, with a prevalence similar to that of atopic dermatitis (AD). 10,11,27 JEB (100%) and RDEB (100%) had the highest prevalence, followed by DDEB (87%) and EBS (74%).…”

Section: Discussionmentioning

confidence: 99%

“…In addition to histamine, a number of non‐histaminergic itch mediators and receptors have recently been identified 20,25 . In EB, the itch is a common symptom with a significant impact on quality of life and is often accompanied by pain and skin infections in the patients 10,11,26 . In a study of the prevalence of itch in 40 adults with either of three EB types (EBS, JEB, or DEB), 85% reported itch, with a prevalence similar to that of atopic dermatitis (AD) 10,11,27 .…”

Section: Discussionmentioning

confidence: 99%

“…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%

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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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“…Improvement was observed from 4 weeks to 12 weeks after dupilumab administration. Six DEB‐Pr patients was reported to benefit from dupilumab treatment 67–71 : total treatment duration differ from 3 months to 22 months with significant improvements were observed in all these six patients. A retrospective study revealed an activation of type 2 immune response including mast cell infiltration, elevated IgE and increased Th2 subset in DEB‐Pr patients, confirming the possible efficacy of dupilumab 66 .…”

Section: Introductionmentioning

confidence: 99%

Dupilumab: Advances in the off‐label usage ofIL4/IL13antagonist in dermatoses

Jia

Zhao

Shi

et al. 2022

Dermatologic Therapy

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Type 2 immune response refers to a complicated series of immune responses characterized by Th2 polarization and Th2 cytokines secretion. The IgE secretion, airway hypersensitivity, and effector cell recruitment (eosinophils, mast cells, basophils) in skin lesion and peripheral blood stream could be upregulated during the activation of type 2 immune response. Th1/Th2 ratio, also referred as Th1/Th2 balance, represent the T lymphocytes immune pattern to a certain degree: Th1-dominated responses are often involved in intracellular infections (e.g., mycobacterium tuberculosis) and autoimmune diseases (e.g., Graves' disease) while Th2-dominated responses are involved in allergic conditions (e.g., atopic dermatitis, eczema), IgE mediated diseases (e.g., urticaria), and fibrotic dermatoses (e.g., keloids). Dupilumab, as one of the most widely applied Th2 cytokine inhibitors, could block the bioactivity of IL-14/IL-13 via competitively binding to the common IL-4Rα subunit shared by IL-4 and IL-13 receptors. In addition to the direct inhibition of type 2 response, dupilumab is also effective in autoimmune and some infectious skin diseases through indirect regulation of type 1 immune response. The pathological mechanism of Th2 responses and advanced clinical application of dupilumab in skin diseases will be summarized and discussed in the review.

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A case of dystrophic epidermolysis bullosa pruriginosa treated with dupilumab

Cited by 18 publications

References 8 publications

Pruriginosa Pattern of Dystrophic Epidermolisys Bullosa: Clinical Case

Pruriginosa Pattern of Dystrophic Epidermolisys Bullosa: Clinical Case

New insight of itch mediators and proinflammatory cytokines in epidermolysis bullosa

Dupilumab: Advances in the off‐label usage ofIL4/IL13antagonist in dermatoses

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