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Frontiers in Immunology 2022Pemphigoid diseases are autoimmune chronic inflammatory skin diseases, which are characterized by blistering of the skin and/or mucous membranes, and circulating and... (Review)
Review
Pemphigoid diseases are autoimmune chronic inflammatory skin diseases, which are characterized by blistering of the skin and/or mucous membranes, and circulating and tissue-bound autoantibodies. The well-established pathomechanisms comprise autoantibodies targeting various structural proteins located at the dermal-epidermal junction, leading to complement factor binding and activation. Several effector cells are thus attracted and activated, which in turn inflict characteristic tissue damage and subepidermal blistering. Moreover, the detection of linear complement deposits in the skin is a diagnostic hallmark of all pemphigoid diseases. However, recent studies showed that blistering might also occur independently of complement. This review reassesses the importance of complement in pemphigoid diseases based on current research by contrasting and contextualizing data from , murine and human studies.
Topics: Animals; Autoantibodies; Blister; Complement System Proteins; Humans; Mice; Pemphigoid, Bullous; Skin
PubMed: 36059476
DOI: 10.3389/fimmu.2022.973702 -
Actas Dermo-sifiliograficas May 2022
Topics: Blister; Humans; Pemphigoid, Bullous
PubMed: 35697410
DOI: 10.1016/j.ad.2020.10.009 -
CMAJ : Canadian Medical Association... Feb 2022
Topics: Administration, Oral; Amoxicillin; Anti-Bacterial Agents; Blister; Child, Preschool; Diagnosis, Differential; Female; Foot Dermatoses; Humans; Staphylococcal Infections; Staphylococcus aureus
PubMed: 35131755
DOI: 10.1503/cmaj.210685 -
Frontiers in Immunology 2023
Topics: Humans; Autoimmune Diseases; Blister
PubMed: 37006261
DOI: 10.3389/fimmu.2023.1175962 -
British Journal of Biomedical Science 2023Autoimmune blistering diseases (AIBD) comprise a heterogeneous group of uncommon disorders of the skin and mucous membranes, characterised by antibodies targeting... (Review)
Review
Autoimmune blistering diseases (AIBD) comprise a heterogeneous group of uncommon disorders of the skin and mucous membranes, characterised by antibodies targeting structural proteins within epithelial tissue and the underlying basement membrane. There can be significant overlap in clinical presentation of these diseases and accurate diagnosis relies on the detection and characterisation of relevant autoantibodies. Immunofluorescence provides the gold-standard diagnostic tool for these diseases, identifying both tissue-bound autoantibodies in biopsy material using direct immunofluorescence and circulating antibodies in serum through indirect immunofluorescence. Following advances in the identification and subsequent characterisation of numerous antigenic targets in these diseases, the development of antigen-specific tests, in particular, enzyme-linked immunosorbent assays on serum specimens, has provided a third key tool to not only identify, but also quantify AIBD autoantibodies. This quantification has proven particularly useful in monitoring disease activity and informing clinical management decisions. Accurate diagnosis of these diseases is important since optimal treatment strategies differ between them and, prognostically, some diagnoses are associated with an increased risk of malignancy. This review outlines the molecular pathology underlying the major AIBD and describes how the three principal techniques can be used in combination, to provide best practice for diagnosis and treatment monitoring.
Topics: Humans; Autoimmune Diseases; Blister; Autoantibodies; Enzyme-Linked Immunosorbent Assay
PubMed: 38074463
DOI: 10.3389/bjbs.2023.11809 -
The Journal of Investigative Dermatology May 2016
Review
Topics: Adolescent; Blister; DNA Methylation; Epidermolysis Bullosa; Female; Genetic Predisposition to Disease; Germ-Line Mutation; Humans; Melanoma; Nevus, Pigmented; PTEN Phosphohydrolase; Periodontal Diseases; Photosensitivity Disorders; Proto-Oncogene Proteins B-raf; Sensitivity and Specificity; Skin Neoplasms
PubMed: 27140323
DOI: 10.1016/j.jid.2016.03.007 -
CMAJ : Canadian Medical Association... Aug 2021
Topics: Aged, 80 and over; Blister; Female; Fondaparinux; Humans; Skin Diseases
PubMed: 34426453
DOI: 10.1503/cmaj.202747-f -
Orphanet Journal of Rare Diseases Sep 2021Epidermolysis bullosa (EB) is characterized by skin fragility with blister formation occurring spontaneously or following minor trauma such as gentle pressure or... (Review)
Review
Epidermolysis bullosa (EB) is characterized by skin fragility with blister formation occurring spontaneously or following minor trauma such as gentle pressure or friction. Current physiotherapy practice is based on anecdotal care, clinical expertise and creative problem solving with caregivers and individuals with EB. Evidence based intervention is needed to establish a foundation of knowledge and to guide international practitioners to create and improve standards of care to effectively work with individuals living with EB. This clinical practice guideline (CPG) was created for the purpose of providing evidence based interventions and best clinical practices for the physiotherapy management of individuals with EB. A survey was conducted within the EB community and six outcomes were identified as a priority to address in physiotherapy management, including (1) attaining developmental motor milestones, (2) identifying safe and functional mobility in the natural environment, (3) encouraging ambulation endurance, (4) supporting safe ability to bear weight, (5) improving access to physiotherapy services, and (6) optimizing interaction with the community. A systematic literature review was conducted and articles were critically analyzed by an international panel consisting of thirteen members: healthcare professionals (including physiotherapist, doctors, and occupational therapist), caregivers, and individuals with EB. Recommendations were formulated from evidence and panel consensus. An external panel of twelve were invited to improve the quality and gather feedback on draft manuscript and recommendations. This CPG describes the development of recommendations for physiotherapy management including several best practice interventions. This guideline lays the foundational work for physiotherapist throughout the world to provide high quality services while improving and maintaining functional mobility and independence within the EB community. The CPG outlines limitations in the evidence available and possible future research needed to improve physiotherapy practice.
Topics: Blister; Epidermolysis Bullosa; Humans; Medicine; Physical Therapy Modalities; Physicians
PubMed: 34593011
DOI: 10.1186/s13023-021-01997-w -
Dermatology Online Journal Nov 2019Neonatal sucking blisters result from vigorous sucking on hand or forearm in utero. Clinically, one observes a tense, fluid-filled blister, which when ruptured forms an...
Neonatal sucking blisters result from vigorous sucking on hand or forearm in utero. Clinically, one observes a tense, fluid-filled blister, which when ruptured forms an erosion. We report a female neonate with a sucking blister on the distal dorsal aspect of her left forearm. These benign bullae should be differentiated from other diseases of the newborn through their presentation, characteristic morphology, and self-limiting course.
Topics: Blister; Diagnosis, Differential; Female; Fetus; Forearm; Humans; Infant, Newborn; Sucking Behavior
PubMed: 32045151
DOI: No ID Found -
Frontiers in Immunology 2022Pemphigus vulgaris (PV) is an autoimmune bullous skin disease caused primarily by autoantibodies (PV-IgG) against the desmosomal adhesion proteins desmoglein (Dsg)1 and... (Review)
Review
Pemphigus vulgaris (PV) is an autoimmune bullous skin disease caused primarily by autoantibodies (PV-IgG) against the desmosomal adhesion proteins desmoglein (Dsg)1 and Dsg3. PV patient lesions are characterized by flaccid blisters and ultrastructurally by defined hallmarks including a reduction in desmosome number and size, formation of split desmosomes, as well as uncoupling of keratin filaments from desmosomes. The pathophysiology underlying the disease is known to involve several intracellular signaling pathways downstream of PV-IgG binding. Here, we summarize our studies in which we used transmission electron microscopy to characterize the roles of signaling pathways in the pathogenic effects of PV-IgG on desmosome ultrastructure in a human skin model. Blister scores revealed inhibition of p38MAPK, ERK and PLC/Ca to be protective in human epidermis. In contrast, inhibition of Src and PKC, which were shown to be protective in cell cultures and murine models, was not effective for human skin explants. The ultrastructural analysis revealed that for preventing skin blistering at least desmosome number (as modulated by ERK) or keratin filament insertion (as modulated by PLC/Ca) need to be ameliorated. Other pathways such as p38MAPK regulate desmosome number, size, and keratin insertion indicating that they control desmosome assembly and disassembly on different levels. Taken together, studies in human skin delineate target mechanisms for the treatment of pemphigus patients. In addition, ultrastructural analysis supports defining the specific role of a given signaling molecule in desmosome turnover at ultrastructural level.
Topics: Acantholysis; Animals; Blister; Desmosomes; Humans; Immunoglobulin G; Keratins; Mice; Pemphigus; p38 Mitogen-Activated Protein Kinases
PubMed: 35720332
DOI: 10.3389/fimmu.2022.884067