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Dupilumab in a 9-week-old with Netherton Syndrome Leads to Deep Symptom Control.
J Clin Immunol
November 2024
Department of Pediatrics, Dr von Hauner Children's Hospital, Ludwig-Maximilians-University Munich, Lindwurmstraße 4 , Munich, European Union (EU), D-80337 , Germany.
Purpose: Netherton syndrome (NS) is a rare inborn error of immunity (IEI) with an incidence of approximately 1:200,000 and the phenotypic triad of trichorrhexis invaginate (bamboo hair), congenital ichthyosiform erythroderma, and multiple atopic manifestations. Treatment options especially in infants are scarce and generally not licensed.
Methods: Case report of a 9-week-old infant with NS treated with dupilumab off-label.
Rapid identification of primary atopic disorders (PAD) by a clinical landmark-guided, upfront use of genomic sequencing.
Allergol Select
October 2024
Center for Child and Adolescent Health, Helios Hospital Krefeld, Academic Hospital of RWTH Aachen, Krefeld.
Article Synopsis
- Primary atopic disorders (PAD) are rare genetic conditions caused by specific gene variants that affect skin and immune function, making diagnosis challenging among common allergic disease cases.
- Identifying PAD requires recognizing clinical red flags like family history and unusual infections, as conventional lab tests are inadequate for definitive diagnosis.
- Whole-genome sequencing (WGS) enhances diagnostic efficiency and accuracy, but requires careful interpretation and collaboration among specialists to effectively manage PAD cases.
Pediatric Dermatitis Seborrhoica - A Clinical and Therapeutic Review.
Indian Dermatol Online J
April 2024
Department of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital, Dresden, Germany.
Pediatric dermatitis seborrhoica (DS) is a common inflammatory disorder of infancy and adolescence distinct from atopic dermatitis. We performed a narrative review on clinical and therapeutic aspects of the disease. The prevalence varies geographically and can reach up to 10%.
View Article and Find Full Text PDFKey Clinical Message: High-dose intravenous immunoglobulin exhibits great potential in the treatment of Netherton syndrome.
Abstract: Netherton syndrome (NS) is a rare autosomal recessive genodermatosis (OMIM #256500) characterized by superficial scaling, atopic manifestations, and multisystemic complications. It is caused by loss-of-function mutations in the SPINK5 gene, which encode a key kallikrein protease inhibitor.
Erythrodermic Presentation of Atopic Dermatitis in a Patient with Secondary Adrenal Insufficiency Caused by Oral Glucocorticosteroid Abuse.
Acta Dermatovenerol Croat
November 2023
Alicja Mesjasz, Dermatological Students Scientific Association, Department of Dermatology, Venereology and Allergology, Faculty of Medicine, Medical University of GdanskFaculty of Medicine, Medical University of Gdansk, Gdansk 80-214, Gdansk, Poland;
Dear Editor, A 41-year-old man presented to the Department of Dermatology for the first time due to an exacerbation of atopic dermatitis (AD) in the form of erythroderma. The patient had a history of atopic diseases, with being AD active from infancy. On clinical examination, generalized erythematous skin lesions causing acute pruritus and accompanied by severe skin exfoliation and dryness were present.
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