Search results for "epidermolysis bullosa"

T cells mediate autoantibody-induced cutaneous inflammation and blistering in epidermolysis bullosa acquisita

2016

AbstractT cells are key players in autoimmune diseases by supporting the production of autoantibodies. However, their contribution to the effector phase of antibody-mediated autoimmune dermatoses, i.e., tissue injury and inflammation of the skin, has not been investigated. In this paper, we demonstrate that T cells amplify the development of autoantibody-induced tissue injury in a prototypical, organ-specific autoimmune disease, namely epidermolysis bullosa acquisita (EBA) – characterized and caused by autoantibodies targeting type VII collagen. Specifically, we show that immune complex (IC)-induced inflammation depends on the presence of T cells – a process facilitated by T cell receptor (…

Plectin-related scapuloperoneal myopathy with treatment-responsive myasthenic syndrome

2020

Placement of endosseous implants in patients with oral epidermolysis bullosa.

2000

Abstract Objective: To evaluate the feasibility of placing endosseous implants in patients with recessive dystrophic epidermolysis bullosa. This article reports on 4 patients subjected to follow-up. Study Design: Implants were placed in 4 cases. All patients have marked oral involvement, with devastating alterations in the soft and hard tissues in all cases. Results: A total of 15 implants (7 maxillary, 8 mandibular) were placed. All implants integrated successfully and have been restored. The average follow-up from implant placement was 2.5 years (range, 1-4). Conclusions: Our preliminary findings suggest that endosseous implants can be successfully placed and can provide support for prost…

Clinical practice guidelines: Oral health care for children and adults living with epidermolysis bullosa.

2020

Background Inherited epidermolysis bullosa (EB) is a genetic disorder characterized by skin fragility and unique oral features. Aims To provide (a) a complete review of the oral manifestations in those living with each type of inherited EB, (b) the current best practices for managing oral health care of people living with EB, (c) the current best practices on dental implant-based oral rehabilitation for patients with recessive dystrophic EB (RDEB), and (d) the current best practice for managing local anesthesia, principles of sedation, and general anesthesia for children and adults with EB undergoing dental treatment. Methods Systematic literature search, panel discussion including clinical…

Fixed Implant-Supported Full-Arch Prosthesis in Epidermolysis Bullosa With Severe Symptoms

2016

Oral rehabilitation with dental implants in patients with recessive dystrophic epidermolysis bullosa : a retrospective study with 2-15 years of follo…

2019

Background Epidermolysis bullosa (EB) comprises a group of hereditary disorders characterized by mechanical fragility of the skin and mucous membranes, with the development of blisters and vesicles in response to minimum tissue friction. Recessive dystrophic epidermolysis bullosa (RDEB) with generalized involvement is the most common subtype in the oral cavity. The present study was carried out to investigate dental implant survival, peri-implant tissue condition, patient satisfaction, and the impact of treatment upon the quality of life of patients with RDEB rehabilitated with implants and full-arch implant-supported prostheses. Material and Methods Thirteen patients with RDEB underwent de…

Structural and regulatory functions of keratins.

2007

The diversity of epithelial functions is reflected by the expression of distinct keratin pairs that are responsible to protect epithelial cells against mechanical stress and to act as signaling platforms. The keratin cytoskeleton integrates these functions by forming a supracellular scaffold that connects at desmosomal cell-cell adhesions. Multiple human diseases and murine knockouts in which the integrity of this system is destroyed testify to its importance as a mechanical stabilizer in certain epithelia. Yet, surprisingly little is known about the precise mechanisms responsible for assembly and disease pathology. In addition to these structural aspects of keratin function, experimental e…

Dental implants in patients with oral mucosal alterations : an update

2010

Objective: To determine whether a series of diseases of the oral mucosa - Sjogren syndrome, ectodermal dysplasia, epidermolysis bullosa and lichen planus - reduce the survival rate of dental implants. Material and Method: A Medline search was carried out using the key words: "Sjogren syndrome", "ectodermal dysplasia", "epidermolysis bullosa", "lichen planus" and "dental implants", including those publications involving clinical series comprising more than one patient with the mentioned disorders and treated with dental implants, in the last 10 years. Results: The study included three articles involving patients with Sjogren syndrome subjected to dental implant treatment, representing a tota…

Gesteigerte fibrinolytische Aktivit�t im Blut bei Epidermolysis bullosa hereditaria

1969

Bei 7 Kranken mit Epidermolysis bullosa hereditaria und 3 hauterscheinungsfreien Familienmitgliedern wurde der Gerinnungsstatus uberpruft. Es ergaben sich bei 9 von 10 der Untersuchten eine verkurzte Euglobulinlysezeit als Ausdruck einer gesteigerten fibrinolytischen Aktivitat im Blut, jedoch keine Storungen des Coagulationssystems. Die Verbindung zwischen diesem humoralen Befund und der Phanomenologie der hereditaren Epidermolysen an der Haut wird diskutiert.

Ectopic expression of desmin in the epidermis of transgenic mice permits development of a normal epidermis.

2002

Cell architecture is largely based on the interaction of cytoskeletal proteins, which include intermediate filaments (IF), microfilaments, microtubules, as well as their type-specific membrane-attachment structures and associated proteins. In order to further our understanding of IF proteins and to address the fundamental issue whether different IF perform unique functions in different tissues, we expressed a desmin transgene in the basal epidermis of mice. Ectopic expression of desmin led to the formation of an additional, keratin-independent IF cytoskeleton and did not interfere with the keratin-desmosome interaction. We show that ectopic expression of a type III IF protein in basal kerat…