Article

Hyper IgE Syndrome Related Disease Treated with Dupilumab: A Case Report

2023-02-15

Abstract excerpt

We describe a case of a 2-month-old female displaying clinical phenotype of HIES related disease with severe eczematous dermatitis recalcitrant to corticosteroids. Genetic panel showed a variant of unknown significance in key immune regulator of PGM3. Patient achieved nearly complete response from off-label use of dupilumab.

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: e78a4513-2997-555d-bcaa-66936852fa45
DOI: 10.22541/au.167645531.10633138/v1

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Hyper IgE Syndrome Related Disease Treated with Dupilumab: A Case ReportDOI 10.22541/au.167645531.10633138/v1Read

Clinical features, genetics, treatment, and long-term outcomes of STAT3 hyper-IgE syndrome: Single-center cohort analysis.Semantic proximity Read

Diagnostic challenge in a series of eleven patients with hyper IgE syndromes.Semantic proximity Read

Clearance of atypical cutaneous manifestations of hyper-IgE syndrome with dupilumab.Semantic proximity Read

Relieving job: Dupilumab in autosomal dominant STAT3 hyper-IgE syndrome.Semantic proximity Read

Identification of a novel COL7A1 variant associated with dystrophic epidermolysis bullosa pruriginosa responding effectively to dupilumab.Semantic proximity Read

Clinical Manifestation of Hyper IgE Syndrome Including Otitis Media.Semantic proximity Read

An Update on Syndromes with a Hyper-IgE PhenotypeSemantic proximity Read

Unravelling the mystery: severe dermatitis, multiple allergies and metabolic wasting (SAM) syndrome with a novel homozygous mutation in DSG1.Semantic proximity Read

Generalized eczematous dermatitis and pruritus responsive to dupilumab in a patient with immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome.Semantic proximity Read

Clinical Profile of Hyper-IgE Syndrome in India.Semantic proximity Read

Genetic profiling of patients with atopic dermatitis reveals immune and skin barrier variants associated with generalized eczema and optimal response to Dupilumab therapy.Semantic proximity Read

Pediatric IPEX-Associated Dermatitis Responds To Dupilumab: Evidence from Skin Transcriptomics and Immune Profiling.Semantic proximity Read