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Hailey-Hailey disease successfully treated with naloxone: 2 case reports and Review of the literature on efficacy of opioid receptor antagonist in Hailey-Hailey disease patients.
J Dermatolog Treat
December 2025
Hospital for Skin Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, Jiangsu, China.
Background: Hailey-Hailey disease (HHD), a genetic blistering disease, is caused by a mutation in a calcium transporter protein in the Golgi apparatus encoded by the gene. Clinically, HHD is characterized by flaccid vesicles, blisters, erosions, fissures, and maceration mainly in intertriginous regions. Some patients remain refractory to conventional treatments.
View Article and Find Full Text PDFMeeting report - Alpine desmosome disease meeting 2024: advances and emerging topics in desmosomes and related diseases.
J Cell Sci
January 2025
Institute of Anatomy and Experimental Morphology, Center for Experimental Medicine, University Cancer Center Hamburg, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany.
Desmosomes are adhesive cell contacts abundant in tissues exposed to mechanical strain, such as the stratified and simple epithelia of the epidermis and mucous membranes, as well as the myocardium. Besides their role in mechanical cell cohesion, desmosomes also modulate pathways important for tissue differentiation, wound healing and immune responses. Dysfunctional desmosomes, resulting from pathogenic variants in genes encoding desmosomal components, autoantibodies targeting desmosomal adhesion molecules or inflammation, cause the life-threatening diseases arrhythmogenic cardiomyopathy and pemphigus and contribute to the pathogenesis of inflammatory bowel diseases.
View Article and Find Full Text PDFEffective treatment of recalcitrant Hailey-Hailey disease with dupilumab.
JAAD Case Rep
January 2025
Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin.
Treatment-related adrenal insufficiency in patients with autoimmune blistering diseases.
JAAD Int
February 2025
Department of Dermatology, Changi General Hospital, Singapore.
Case report of the successful treatment of pemphigus vulgaris using ovine forestomach matrix graft.
J Surg Case Rep
January 2025
Department of Medical Affairs, Aroa Biosurgery Limited, 64 Richard Pearse Drive, Auckland 2022, New Zealand.
Pemphigus vulgaris (PV) is a subtype of pemphigus and life-altering disorder that results in the formation of intraepithelial blisters in mucosa and skin. Though the etiology is not well understood, it is an autoimmune disorder resulting in acantholytic blisters due to auto-antibodies targeting proteins of keratinocyte adhesion. Rapid diagnosis and restoration of the epidermal layer is imperative for patients with PV as widespread epidermal damage can lead to high morbidity and mortality rates.
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