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| http://dx.doi.org/10.4269/ajtmh.20-0736 | DOI Listing |
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A Novel COL7A1 Mutation in a Patient With Dystrophic Epidermolysis Bullosa. Successful Treatment With Upadacitinib.
Clin Cosmet Investig Dermatol
January 2025
Department of Dermatology, Candidate Branch of National Clinical Research Centre for Skin and Immune Diseases, First Affiliated Hospital of Gannan Medical University, Ganzhou, 341000, People's Republic of China.
Dystrophic epidermolysis bullosa (DEB) is a heterogeneous and rare genetic skin disease caused by mutations in the gene, which encodes Type VII collagen. The absence or dysfunction of Type VII collagen can cause the dense lower layer of the basal membrane zone of the skin to separate from the dermis, leading to blister formation and various complications. In different DEB subtypes, the severity of the phenotype is associated, to some extent, with the outcome of Type VII collagen caused by mutations in the gene, which may be reduced in expression, remarkably reduced, or completely absent.
View Article and Find Full Text PDFPreliminary experimental study on a novel device using biomaterial for shunting fibrotic bleb following Ahmed glaucoma valve surgery in dogs.
Sci Rep
January 2025
Department of Veterinary Clinical Sciences, College of Veterinary Medicine and Research Institute for Veterinary Science, Seoul National University, Seoul, Republic of Korea.
This study aimed to design and evaluate a novel trans-bleb device (TBD) for draining aqueous humor trapped within fibrotic blebs following Ahmed glaucoma valve (AGV) implantation in dogs. Two clinically normal, purpose-bred Beagles underwent AGV implantation surgery in one eye. When a bleb was formed with increased intraocular pressure (IOP), the TBD was inserted through a fenestration created in the bleb.
View Article and Find Full Text PDFDyshidrosiform Bullous Pemphigoid: A Palmar Variant of a Common Disease.
Cureus
December 2024
Dermatology, Corewell Health Farmington Hills Hospital, Farmington Hills, USA.
Dyshidrosiform bullous pemphigoid (DBP) is a rare variant of bullous pemphigoid (BP) that mainly affects elderly patients and presents with tense bullae formation on the palms, soles, or both palms and soles. This case report describes an 87-year-old woman who was evaluated in the hospital for a month-long erythematous and pruritic rash on most of her body that eventually manifested into tense blisters on the palms. DBP can pose a challenge to clinicians as it can resemble a variety of different vesicular diseases.
View Article and Find Full Text PDFImmune checkpoint inhibitor-induced severe epidermal necrolysis mediated by macrophage-derived CXCL10 and abated by TNF blockade.
Nat Commun
December 2024
Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou Branch, Taoyuan, Taiwan.
Immune checkpoint inhibitors (ICI) represent new anticancer agents and have been used worldwide. However, ICI can potentially induce life-threatening severe cutaneous adverse reaction (SCAR), such as Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), hindering continuous ICI therapy. We examine 6 cohorts including 25 ICI-induced SJS/TEN patients and conduct single-cell RNA sequencing (scRNA-seq) analysis, which shows overexpression of macrophage-derived CXCL10 that recruits CXCR3 cytotoxic T lymphocytes (CTL) in blister cells from ICI-SJS/TEN skin lesions.
View Article and Find Full Text PDFSuccessful Treatment of Immune Checkpoint Inhibitor-Induced Bullous Pemphigoid with Omalizumab: A Case Report and Review of the Literature.
Clin Cosmet Investig Dermatol
December 2024
Department of Dermatology, Guangzhou Dermatology Hospital, Guangzhou, 510095 People's Republic of China.
Background: Immune checkpoint inhibitors (ICIs) have revolutionized cancer treatment by enhancing the immune system's ability to target cancer cells. However, ICIs can lead to immune-related adverse events (irAEs), including dermatologic manifestations such as bullous pemphigoid (BP).
Objective: To evaluate the efficacy and safety of omalizumab and other biologics in the treatment of ICI-induced refractory bullous pemphigoid and to derive a strategy for selecting biologic treatments for this condition.
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