Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11719851PMC
http://dx.doi.org/10.1016/j.jdin.2024.11.002DOI Listing

Publication Analysis

Top Keywords

recalcitrant hailey-hailey
4
hailey-hailey disease
4
disease treated
4
treated isotretinoin
4
isotretinoin family
4
family case
4
case series
4
recalcitrant
1
disease
1
treated
1

Similar Publications

Long-term remission of Hailey-Hailey disease by Er:YAG ablative laser therapy.

J Eur Acad Dermatol Venereol

October 2024

Department of Dermatology, Centre of Expertise for Genodermatoses, Maastricht University Medical Centre+, Maastricht, The Netherlands.

Background: Hailey-Hailey disease (HHD) is a rare genetic therapy-resistant blistering disease with great disease burden. Treatment is currently focused on symptomatic relief. Er:YAG ablative laser therapy is a therapeutic modality with promising results, though evidence is currently scarce.

View Article and Find Full Text PDF
Article Synopsis
  • - Hailey-Hailey disease is an autosomal dominant condition caused by a mutation in the ATP2C1 gene, leading to painful skin blisters and erosions, particularly in folds of the skin.
  • - There is currently no cure for the disease, and treatments mainly focus on managing infections and inflammation, with a recent emphasis on targeting the disease's biochemical pathways.
  • - In a case series involving three patients with severe Hailey-Hailey disease, the use of the biologic drug adalimumab successfully controlled their symptoms and maintained remission.
View Article and Find Full Text PDF

This report presents two cases of patients with long-standing, treatment-resistant Hailey-Hailey disease (HHD) who experienced significant symptom relief through a combination therapy of oral naltrexone and dupilumab injections. The therapeutic potential of targeting the Th2 pathway and Ca signaling with dupilumab in managing HHD manifestations is highlighted. The findings suggest that Th2 blockade with dupilumab, in conjunction with naltrexone, effectively controls recalcitrant HHD, indicating a role of cytokine response in altering disease pathogenesis.

View Article and Find Full Text PDF

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!