Dupilumab to treat severe atopic dermatitis in autosomal dominant hyper-IgE syndrome

Sogkas, Georgios; Hirsch, Stefanie; Jablonka, Alexandra; Witte, Torsten; Schmidt, Reinhold; Atschekzei, Faranaz

doi:10.1016/j.clim.2020.108452

Cited by 32 publications

(28 citation statements)

References 7 publications

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“…25 Dupilumab treatment was previously reported in two adult patients with AD-HIES with STAT3 mutation for severe dermatitis with good results and safety profile. 11,12 Treatment with dupilumab has been found useful and safe for complications, and any opportunity to bring patients to transplant with less infection and inflammation would hopefully reduce morbidity in the transplant procedure.…”

Section: Discussionmentioning

confidence: 99%

“…14 In cases of AD-HIES, dermatitis improved within 3-4 weeks of treatment and the pruritus was reported to improve within 2 weeks. 11 IgG4 related dermatitis and asthma improved within 3 months of treatment with dupilumab. 14 This is the first report of successful treatment of two children with dupilumab for severe recalcitrant dermatitis related to DOCK8 deficiency.…”

Section: Introductionmentioning

confidence: 92%

“…Dupilumab treatment was previously reported in adult patients with immunodeficiency-related dermatitis, including patients with AD-HIES due to STAT3 mutation, 11,12 common variable immunodeficiency, 13 and IgG4-related disease. 14 In cases of AD-HIES, dermatitis improved within 3-4 weeks of treatment and the pruritus was reported to improve within 2 weeks.…”

Section: Introductionmentioning

confidence: 99%

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Treatment options for DOCK8 deficiency‐related severe dermatitis

Ollech

Mashiah

Lev

et al. 2021

The Journal of Dermatology

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 92%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Treatment options for DOCK8 deficiency‐related severe dermatitis

Ollech

Mashiah

Lev

et al. 2021

The Journal of Dermatology

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“…Pneumonia is common, and predisposes to formation of bronchiectasis and pneumatoceles [123,124] Consider antifungal prophylaxis with mold-active azoles such as itraconazole in patients with parenchymal disease (bronchiectasis, pneumatocele)…”

Section: Recurrent Pulmonary Infection Offer Twice-daily Co-trimoxazole Prophylaxismentioning

confidence: 99%

STAT3 Hyper-IgE Syndrome—an Update and Unanswered Questions

2021

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The hyper-IgE syndromes (HIES) are a heterogeneous group of inborn errors of immunity sharing manifestations including increased infection susceptibility, eczema, and raised serum IgE. Since the prototypical HIES description 55 years ago, areas of significant progress have included description of key disease-causing genes and differentiation into clinically distinct entities. The first two patients reported had what is now understood to be HIES from dominant-negative mutations in signal transduction and activator of transcription 3 (STAT3-HIES), conferring a broad immune defect across both innate and acquired arms, as well as defects in skeletal, connective tissue, and vascular function, causing a clinical phenotype including eczema, staphylococcal and fungal skin and pulmonary infection, scoliosis and minimal trauma fractures, and vascular tortuosity and aneurysm. Due to the constitutionally expressed nature of STAT3, initial reports at treatment with allogeneic stem cell transplantation were not positive and treatment has hinged on aggressive antimicrobial prophylaxis and treatment to prevent the development of end-organ disease such as pneumatocele. Research into the pathophysiology of STAT3-HIES has driven understanding of the interface of several signaling pathways, including the JAK-STAT pathways, interleukins 6 and 17, and the role of Th17 lymphocytes, and has been expanded by identification of phenocopies such as mutations in IL6ST and ZNF341. In this review we summarize the published literature on STAT3-HIES, present the diverse clinical manifestations of this syndrome with current management strategies, and update on the uncertain role of stem cell transplantation for this disease. We outline key unanswered questions for further study.

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“…The monoclonal antibody dupilumab targets the Th‐2 immune response in AD through blocking the IL‐4 receptor. Successful treatment using dupilumab has been reported in two cases of hyper‐IgE syndrome related to ZNF431 mutations 72,73 . The over‐production of IgE is also a promising target for diseases within the hyper‐IgE spectrum.…”

Section: Immune Deficiencies With Atopic Phenotypesmentioning

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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Dupilumab to treat severe atopic dermatitis in autosomal dominant hyper-IgE syndrome

Cited by 32 publications

References 7 publications

Treatment options for DOCK8 deficiency‐related severe dermatitis

Treatment options for DOCK8 deficiency‐related severe dermatitis

STAT3 Hyper-IgE Syndrome—an Update and Unanswered Questions

Genodermatoses with itch as a prominent feature

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