Dupilumab for severe generalized eczematous eruption complicating common variable immunodeficiency

Votquenne, Nicolas; Dupire, Gwendy; Michel, Olivier; Saïd, B. Ben

doi:10.1684/ejd.2020.3954

Cited by 4 publications

(4 citation statements)

References 6 publications

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“…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%

Treatment options for DOCK8 deficiency‐related severe dermatitis

Ollech

Mashiah

Lev

et al. 2021

The Journal of Dermatology

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Section: Introductionmentioning

confidence: 99%

Treatment options for DOCK8 deficiency‐related severe dermatitis

Ollech

Mashiah

Lev

et al. 2021

The Journal of Dermatology

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“…Previous studies also support dupilumab treatment of atopic dermatitis in non- STAT3 -related-IEI, such as DOCK8 deficiency ( 40 ), CARD11-associated atopy with dominant interference of NF-kB signaling (CADINS) ( 41 ), Wiskott-Aldrich Syndrome ( 42 ), common variable immune deficiency (CVID) ( 43 ), TTC7A-associated combined immunodeficiency ( 44 ), and X-linked agammaglobulinemia ( 45 – 47 ). Thus, indications for dupilumab use in IEI are rapidly expanding, and it should be considered a main therapeutic modality in adult patients with AD-HIES along with standard topical care.…”

Section: Discussionmentioning

confidence: 77%

Dominant-negative signal transducer and activator of transcription (STAT)3 variants in adult patients: A single center experience

et al. 2022

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BackgroundAutosomal dominant hyper-IgE syndrome (AD-HIES) caused by dominant negative (DN) variants in the signal transducer and activator of transcription 3 gene (STAT3) is characterized by recurrent Staphylococcal abscesses, severe eczema, chronic mucocutaneous candidiasis (CMC), and non-immunological facial and skeletal features.ObjectivesTo describe our experience with the diagnosis and treatment of adult patients with AD-HIES induced by DN-STAT3 variants.MethodsThe medical records of adult patients (>18 years) treated at the Allergy and Clinical Immunology Clinic of Hadassah Medical Center, Jerusalem, Israel, were retrospectively analyzed. Immune and genetic workups were used to confirm diagnosis.ResultsThree adult patients (2 males; age 29-41 years) were diagnosed with DN-STAT3 variants. All patients had non-immunological features, including coarse faces and osteopenia. Serious bacterial infections were noted in all patients, including recurrent abscesses, recurrent pneumonia, and bronchiectasis. CMC and diffuse dermatophytosis were noted in two patients. Two patients had severe atopic dermatitis refractory to topical steroids and phototherapy. Immune workup revealed elevated IgE in three patients and eosinophilia in two patients. Whole exome sequencing revealed DN-STAT3 variants (c.1166C>T; p.Thr389Ile in two patients and c.1268G>A; p. Arg423Gln in one patient). Variants were located in DNA-binding domain (DBD) and did not hamper STAT3 phosphorylation Treatment included antimicrobial prophylaxis with trimethoprim/sulfamethoxazole (n=2) and amoxycillin-clavulanic acid (n=1), and anti-fungal treatment with fluconazole (n=2) and voriconazole (n=1). Two patients who had severe atopic dermatitis, were treated with dupilumab with complete resolution of their rash. No adverse responses were noted in the dupilumab-treated patients.DiscussionDupilumab can be used safely as a biotherapy for atopic dermatitis in these patients as it can effectively alleviate eczema-related symptoms. Immunologists and dermatologists treating AD-HIES adult patients should be aware of demodicosis as a possible manifestation. DN-STAT3 variants in DBD do not hamper STAT3 phosphorylation.

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“…EE of aging is an exclusion diagnosis and was treated with dupilumab leading to sustained improvement of skin lesions and itch in a total of 16 patients without a history of AD or atopic diathesis ( Table 1 ) [ 81 , 82 ]. Furthermore, one record of a patient with EE associated with a common variable immunodeficiency (CVID) demonstrated complete resolution of skin manifestations within 8 weeks [ 83 ]. Secondary generalized eczematization in a patient with underlying chronic venous stasis dermatitis was treated with dupilumab which led to significant improvement of itch; however, new psoriasiform lesions developed subsequently and dupilumab was discontinued [ 84 ].…”

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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Dupilumab for severe generalized eczematous eruption complicating common variable immunodeficiency

Cited by 4 publications

References 6 publications

Treatment options for DOCK8 deficiency‐related severe dermatitis

Treatment options for DOCK8 deficiency‐related severe dermatitis

Dominant-negative signal transducer and activator of transcription (STAT)3 variants in adult patients: A single center experience

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

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