The treatment of refractory atypical bullous pemphigoid with generalized eczema and intense pruritus with dupilumab

Li, Wei; Cai, Sheng; Man, Xiao‐Yong

doi:10.1111/dth.15243

Cited by 8 publications

(4 citation statements)

References 7 publications

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“…This included one pediatric patient [ 117 ]. Efficient dupilumab mono-therapy inducing clinical remission was reported in 3 cases [ 118 , 119 , 120 ]. However, some reports showed only partial or no response [ 114 ].…”

Section: Resultsmentioning

confidence: 99%

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

et al. 2023

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Dupilumab was first approved for the treatment of atopic dermatitis (AD) and blocks the signaling of interleukin (IL)-4 and -13. Several other chronic skin conditions share mechanistic overlaps with AD in their pathophysiology, i.e., are linked to type 2 inflammation. Most recently, dupilumab was approved by the U.S. Food and Drug Administration for prurigo nodularis (PN). Given its relatively good safety profile, effective off-label use of dupilumab has been reported for a multitude of dermatologic diseases and several clinical trials for dermatologic skin conditions are currently ongoing. We conducted a systematic review of applications of dupilumab in dermatology other than AD and PN by searching the databases PubMed/Medline, Scopus, Web of Science and Cochrane Library as well as the clinical trial registry ClinicalTrials.gov. We found several reports for effective treatment of bullous autoimmune diseases, eczema, prurigo, alopecia areata, chronic spontaneous urticaria, Netherton syndrome and a variety of other chronic inflammatory skin diseases.

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Section: Resultsmentioning

confidence: 99%

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

et al. 2023

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“…A multicenter case series and a case-control study have suggested a high disease clearance rate in 12 of 13 (92%) patients and a significantly reduced time of new blister formation in BP patients (6,18). Since then, several case reports have shown dupilumab to be an effective treatment for different subtypes of pemphigoid, including adolescent BP, eczemalike BP, IgA BP, anti-PD-1/PD-L1-induced BP, vesicular pemphigoid, and pemphigoid nodularis (19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32). Dupilumab was suggested in the recently created European guideline for BP management released by the European Academy of Dermatology and Venereology.…”

Section: Resultsmentioning

confidence: 99%

Anti-interleukin 4 receptor α antibody for the treatment of Chinese bullous pemphigoid patients with diverse comorbidities and a 1-year follow-up: a monocentric real-world study

Wang

Shan

et al. 2023

Front. Immunol.

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BackgroundBullous pemphigoid (BP) is a common subepidermal bullous disorder that lacks adequate treatment alternatives. Dupilumab, an anti-interleukin (IL) 4 receptor α antibody blocking Th2 molecules IL-4 and 13, has been used off-label and shown to be effective in refractory BP cases.MethodsBP patients with various disease severities and comorbidities were included in this case series. All patients received dupilumab alone or in combination with immunosuppressants in a real-world setting. Complete remission (CR) was defined as the absence of pruritus symptoms and previous BP eruptions, with only hyperpigmentation patches and without newly occurring lesions for at least 4 weeks. Disease relapse was classified as the appearance of three or more new lesions within 1 month or at least one large urticarial or eczematous lesion that did not resolve within a week.FindingsTen individuals were enrolled in this case series. Pruritus symptoms and BP eruptions improved significantly in nine patients (90%). Seven patients (70%) attained CR, including all mild-to-moderate (100%) cases and three of six (50%) severe BP cases. At the dupilumab monotherapy stage, eosinophilia was observed in two severe cases. One patient out of seven (14.3%) relapsed after 1 year of follow-up after CR.ConclusionTreatment of BP with diverse comorbidities with anti-IL-4 receptor α antibody provides further credentials to a prospective randomized study. More impressive efficacy and safety profiles were observed in patients with mild-to-moderate disease after 1 year of follow-up. Eosinophilia may occur in patients receiving dupilumab monotherapy.

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“…7 Dupilumab is not currently approved for the treatment of BP. However, due to the role of Th2-mediated inflammation in the pathogenesis of BP, research initiatives around the world [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] and within China [24][25][26][27] have used dupilumab to treat refractory or moderate to severe BP. We attempted to use dupilumab to treat 11 BP patients whose underlying diseases made conventional treatment ineffective or who had poor responses to conventional treatment, and we achieved positive results.…”

Section: Discussionmentioning

confidence: 99%

“… 7 Dupilumab is not currently approved for the treatment of BP. However, due to the role of Th2‐mediated inflammation in the pathogenesis of BP, research initiatives around the world 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 and within China 24 , 25 , 26 , 27 have used dupilumab to treat refractory or moderate to severe BP.…”

Section: Discussionmentioning

confidence: 99%

Concomitant use of dupilumab with glucocorticoid in bullous pemphigoid reduces disease severity: A preliminary study

Huang

Fu-qiong

et al. 2023

Immunity Inflam & Disease

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Objective To retrospectively analyze the efficacy and safety of dupilumab in the treatment of bullous pemphigoid. Methods From October 2020 to October 2022, the medical records of patients with bullous pemphigoid who were treated with dupilumab in our department were collected retrospectively to analyze the therapeutic effect and changes in laboratory indexes. Results The records of a total of 11 patients with bullous pemphigoid who were treated with dupilumab was reviewed. Within 2 weeks of the treatment, 10 (90.9%) of the 11 patients had complete or substantial control of the disease. The BPDAI scores of the patients decreased from baseline 113 (62, 181) to 37 (6, 130) at 2 weeks (p = .001) and 4 (0, 37) at 12 weeks after treatment (p < .001). In the 11 patients treated with dupilumab, the relief time of pruritus was 0–3 days (0.5, 7) days, and the pruritus was significantly alleviated after 2 weeks (t = 15.925, p < .001). The DLQI score decreased from (25.5 ± 2.5) before treatment, to (11.8 ± 4.4) at 2 weeks (t = 10.764, p < .001) and (2.1 ± 1.9) at 12 weeks (t = 30.038, p < .001). The patients had high eosinophil counts, high serum IgE levels, low serum total protein levels, and abnormal blood coagulation function. The aforementioned indicators gradually returned to normal after treatment. No adverse reactions occurred during the treatment. Conclusion Dupilumab can effectively control the condition of bullous pemphigoid, efficiently relieve pruritus symptoms, and is relatively safe.

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The treatment of refractory atypical bullous pemphigoid with generalized eczema and intense pruritus with dupilumab

Cited by 8 publications

References 7 publications

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

Anti-interleukin 4 receptor α antibody for the treatment of Chinese bullous pemphigoid patients with diverse comorbidities and a 1-year follow-up: a monocentric real-world study

Concomitant use of dupilumab with glucocorticoid in bullous pemphigoid reduces disease severity: A preliminary study

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