Hyperimmunoglobulin E syndrome (HIES) is a group of rare genetic disorders characterized by severe atopic dermatitis and recurrent skin and pulmonary infections. The efficacy of dupilumab in pediatric patients with HIES-associated severe atopic dermatitis is relatively understudied. Here, we present a series of three children with HIES, two with AD-HIES caused by STAT3 mutations, and one with AR-HIES caused by biallelic mutations in ZNF341. In all cases, dupilumab treatment led to sustained clearance of severe atopic dermatitis over multiple years, as well as improvements in systemic symptoms of HIES.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1111/pde.15780 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Immunohistochemical investigation of transient receptor potential melastatin-2 and spexin immunoreactivity in atopic dermatitis and mycosis fungoides.
Arch Dermatol Res
January 2025
Department of Dermatology, Firat University Hospital, Elazig, TR23119, Turkey.
Background: Atopic dermatitis (AD) is a chronic, pruritic, and inflammatory dermatosis seen in individuals with an atopic predisposition. This study aimed to examine the immunoreactivity of spexin and TRPM2 in skin samples from patients with AD and MF lesions using immunohistochemical methods.
Materials And Methods: The study utilized a total of 60 skin samples, comprising 20 from AD patients, 20 from MF patients, and 20 from control subjects.
[Eosinophilic esophagitis].
Inn Med (Heidelb)
January 2025
Service de gastro-entérologie et d'hepatologie, Centre hospitalier universitaire vaudois (CHUV), Lausanne, Schweiz.
Eosinophilic esophagitis (EoE) was first described in the early 1990s. Initially a rarity, it is now the most common cause of dysphagia for solid foods in young adults. Its prevalence is estimated to be 1:2000.
View Article and Find Full Text PDFCorrection: Factors associated with comorbidity development in atopic dermatitis: a cross-section study.
Arch Dermatol Res
January 2025
The Department of Dermatology, The Second Affiliated Hospital of Dalian Medical University, No. 467 Zhongshan Road, Shahekou District, Dalian, Liaoning, 116000, China.
Editor's Highlights-February 2025.
Int J Dermatol
January 2025
Department of Dermatology and Allergology, University of Szeged, Szeged, Hungary.
Understanding the pathophysiology of inflammatory skin diseases, including psoriasis, atopic dermatitis (AD), and prurigo nodularis (PN), has led to the development of innovative treatments. In the February issue of the Journal, we provide insight into the global epidemiology and psychosocial impact of psoriasis. We also discuss the role of tissue-resident memory T cells in disease recurrence and evaluate the effectiveness of tildrakizumab in treating difficult areas in psoriasis.
View Article and Find Full Text PDFTemporal Relationships Between Staphylococcus aureus Colonization, Filaggrin Expression, and Pediatric Atopic Dermatitis-Correspondence.
Int J Rheum Dis
January 2025
Center for Allergy, Immunology, and Microbiome (AIM), China Medical University Hospital, Taichung, Taiwan.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!