Dupilumab in <scp>Hailey‐Hailey</scp> disease: a case series

Alamón-Reig, Francesc; Serra‐García, Laura; Bosch‐Amate, Xavier; Loughlin, Constanza Riquelme‐Mc; Mascaró, J. M.

doi:10.1111/jdv.18350

Cited by 8 publications

(2 citation statements)

References 8 publications

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“…The most frequent hereditary intraepidermal blistering disease is Hailey–Hailey disease (HHD) often caused by mutations in ATP2C1 that lead to disrupted development of desmosomes of the epidermis and subsequent acantholysis. A total of seven patients were treated with dupilumab after extensive ineffective previous therapies including immunosuppressants, retinoids, antipruritic medications or dermabrasion with significant improvement of skin lesions, itch and quality of life in six patients ( Table 3 ) [ 144 , 145 , 146 ]. One patient did not respond to dupilumab.…”

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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“…6 More recently, reports of significant improvement after treatment with dupilumab within a one-year follow-up period, further suggests that the inhibition of the IL-4 and IL-13 pathways may explain the effectiveness of dupilumab in treating HHD. 7 Alternatively, Licata et al suggest that the skin barrier defect and associated acantholysis seen in HHD promotes local inflammation thus making IL-4 a suitable target for dupilumab. 8 We propose that dupilumab works for HHD due to the significant skin barrier dysfunction, similar to atopic dermatitis, and consider whether inflammation plays an earlier role in the disease.…”

Section: Brief Articlementioning

confidence: 99%

Successful Treatment of Refractory Hailey-Hailey Disease with Dupilumab: A Case Report

King

Wong²,

Potts³

2023

J of Skin

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Hailey-Hailey disease (HHD) is an autosomal dominant blistering dermatosis with incomplete penetrance caused by an ATP2C1 gene mutation. Currently, there is no cure for HHD; however therapeutic options aim to minimize the exacerbating factors and manage patients’ symptoms. A 58-year-old male presented with a 10-year history of biopsy-proven HHD. He was seen consistently over the course of nine years with multiple flares a year consisting of pruritic and painful intertriginous plaques with involvement of the chest, upper arms, and back. His disease was recalcitrant to topicals, oral and topical antibiotics, phototherapy, and systemic corticosteroids. He was started on dupilumab and noticed significant improvement. We present a case of HHD recalcitrant to various modalities of treatment. Our description of rapid improvement with dupilumab suggests a role for Th2 signaling in the pathophysiology of HHD. We propose that dupilumab works for HHD due to the significant skin barrier dysfunction, similar to atopic dermatitis, and consider whether inflammation plays an earlier role in the disease. Although used for an off-label purpose, in this case, further studies should assess the clinical response and safety of patients with recalcitrant HHD treated with dupilumab.

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Hailey-Hailey Disease

Salavastru,

Țiplica

2023

European Handbook of Dermatological Treatments

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Dupilumab in Hailey‐Hailey disease: a case series

Cited by 8 publications

References 8 publications

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

Successful Treatment of Refractory Hailey-Hailey Disease with Dupilumab: A Case Report

Hailey-Hailey Disease

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