نتایج جستجو برای: blistering skin disease
تعداد نتایج: 1643649 فیلتر نتایج به سال:
Mutations in plectin, a widely expressed giant cytolinker protein can lead to different diseases mostly with signs of muscular dystrophy (MD) and skin blistering. The only report of plectin-related disease without skin involvement is limb-girdle muscular dystrophy type 2Q (LGMD2Q) phenotype, showing early-onset limb-girdle muscular dystrophy symptoms with progressive manner and no cranial muscl...
Autoimmune blistering diseases are diverse group of conditions characterized by blisters in the skin with or without mucosal lesions. There may be great degree of clinical and histopathological overlap; hence, advanced immunological tests may be necessary for more precise diagnosis of these conditions. Direct immunofluorescence microscopy is the gold standard tests to demonstrate the tissue-bou...
KP: Koebner phenomenon PV: pemphigus vulgaris INTRODUCTION Pemphigus vulgaris (PV) is a rare autoimmune mucocutaneous blistering disease characterized by epidermal acantholysis, flaccid bullae, and erosive lesions in the skin and mucosa. The Koebner phenomenon (KP) is defined as the appearance of new, typical skin lesions on areas of injury in otherwise healthy skin. KP is described in several ...
BACKGROUND Inherited epidermolysis bullosa (EB) comprises a highly heterogeneous group of rare diseases characterized by fragility and blistering of skin and mucous membranes. Clinical features combined with immunofluorescence antigen mapping and/or electron microscopy examination of a skin biopsy allow to define the EB type and subtype. Molecular diagnosis is nowadays feasible in all EB subtyp...
Background: Epidermolysis bullosa (EB) comprises a group of genetically determined skin fragility disorders, which are characterized by blistering of the skin and mucosa, in response to little or no apparent trauma. These disorders represent heterogeneous phenotypes and are associated with various complications ranging from localized skin fragility to neonatal death. Nevertheless, the term "Epi...
This case report describes the alternative use of an oral endotracheal tube fastener in a pediatric patient with junctional epidermolysis bullosa. The patient underwent dental treatment in the operating room under general anesthesia and had a medical history of junctional epidermolysis bullosa, prior secondary anemia, clubbed feet, and past methicillin-resistant Staphylococcus aureus infection ...
ABStrACt Immunoglobulin A (IgA) represents the most prominent antibody class at mucosal surfaces and plays an important role in mucosal immunity. However, we recently demonstrated that cross-linking of the IgA Fc receptor (FcaRI; CD89) by IgA autoantibodies of patients with autoimmune skin blistering diseases induced massive recruitment of neutrophils, which resulted in severe tissue damage. Cu...
Background Pemphigus is a severe bullous autoimmune skin disease. foliaceus (PF) characterized by antidesmoglein (Dsg) 1 IgG causing epidermal blistering; mucosal pemphigus vulgaris (mPV) anti-Dsg3 inducing erosions in the mucosa; and mucocutaneous (PV) affecting both, with autoantibodies targeting Dsg1 Dsg3. Objectives To characterize Ca2+ flux pathway delineate its importance pathogenesis cli...
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