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Cutis Feb 2021
Topics: Antibodies; Autoantibodies; Dystonin; Enzyme-Linked Immunosorbent Assay; Humans; Pemphigoid, Bullous
PubMed: 33891843
DOI: 10.12788/cutis.0182 -
The Journal of Dermatology Aug 2021Bullous pemphigoid (BP) is an autoimmune skin disease, caused by autoantibodies to BP180 and/or BP230. While both these autoantigens are expressed in the entire skin,...
Bullous pemphigoid (BP) is an autoimmune skin disease, caused by autoantibodies to BP180 and/or BP230. While both these autoantigens are expressed in the entire skin, only some parts of the body become affected. Rare clinical observations indicate that BP may also manifest locally, usually following exposure to triggers. Here, we evaluated the occurrence and potential triggers of localized BP (LBP) in a cohort of 285 BP patients. Medical records of all BP patients hospitalized between 2009 and 2019 were reviewed. In 7/285 BP patients, a localized variant was identified. In 5/7 LBP patients, the disease remained local, while in 2/7 patients, an initial LBP subsequently spread. All cases were preceded by presumptive triggers, including previously described triggers and bacterial infections. Overall, LBP is rare. LBP, however, might be underdiagnosed and should thus be considered in the differential diagnosis, particularly when trigger factors preceded.
Topics: Autoantibodies; Autoantigens; Dystonin; Humans; Non-Fibrillar Collagens; Pemphigoid, Bullous; Prevalence
PubMed: 33998059
DOI: 10.1111/1346-8138.15912 -
International Journal of Dermatology May 2022
Topics: Autoantibodies; Autoantigens; Dystonin; Enzyme-Linked Immunosorbent Assay; Humans; Laminin; Non-Fibrillar Collagens; Pemphigoid, Bullous; Sitagliptin Phosphate
PubMed: 34196971
DOI: 10.1111/ijd.15762 -
Journal of Cutaneous Medicine and... Nov 2016Itching nodules and papules are common findings. A rare but important differential diagnosis is the nodular subtype of bullous pemphigoid.
INTRODUCTION
Itching nodules and papules are common findings. A rare but important differential diagnosis is the nodular subtype of bullous pemphigoid.
METHODS AND RESULTS
The investigators report a female patient presenting with strongly itching papules disseminated over her extremities and trunk. Physical examination revealed multiple erythematous, mostly excoriated papules and nodules on her back, abdomen, and extremities. Histology showed changes compatible with prurigo lesion, and immunofluorescence showed positive results for BP180 and BP230. Considering these clinical, histologic, and immunofluorescence findings, the diagnosis of a nodular subtype of bullous pemphigoid was made. The patient showed healing of lesions under a combination therapy with systemic psoralen and ultraviolet A, topical application of corticosteroids, and systemic therapy with azathioprine and prednisolone.
DISCUSSION
Pemphigoid nodularis represents the rare prurigo variant of bullous pemphigoid. Typically, lesions show the same immunopathologic and histologic features as in common bullous pemphigoid but mostly without the characteristic clinical finding of bullae.
Topics: Aged, 80 and over; Autoantigens; Dystonin; Female; Humans; Non-Fibrillar Collagens; Pemphigoid, Bullous; Pruritus; Collagen Type XVII
PubMed: 27207351
DOI: 10.1177/1203475416648322 -
Archives of Dermatological Research Apr 1999BP180 is a member of the collagen protein family and is also referred to as type XVII collagen or BP antigen 2. It is a transmembrane protein constituent of the... (Review)
Review
BP180 is a member of the collagen protein family and is also referred to as type XVII collagen or BP antigen 2. It is a transmembrane protein constituent of the dermal-epidermal anchoring complex. The long-held hypothesis that BP180 functions as a cell-matrix adhesion molecule has been supported by recent investigations of human disorders of the dermal-epidermal junction in which BP180 is either genetically defective or targeted by the immune system. In generalized atrophic benign epidermolysis bullosa, mutations of BP180 result in an inherited subepidermal blistering disease. In bullous pemphigoid, herpes/pemphigoid gestationis, cicatricial pemphigoid, lichen planus pemphigoides and linear IgA disease, autoantibodies are directed to different epitopes on the BP180 ectodomain. Recent molecular investigations have provided new insights, not only into the mechanism of autoantibody-mediated subepidermal blistering, but also into the biochemical structure and cell biological functions of BP180 and other components of the dermal-epidermal anchoring complex. These findings have suggested new directions for the development of diagnostic and therapeutic tools for these autoimmune and genetic diseases.
Topics: Autoantigens; Carrier Proteins; Collagen; Cytoskeletal Proteins; Dystonin; Epidermolysis Bullosa, Junctional; Humans; Nerve Tissue Proteins; Non-Fibrillar Collagens; Skin; Collagen Type XVII
PubMed: 10335914
DOI: 10.1007/s004030050392 -
Collagen and Related Research Dec 1987
Review
Topics: Autoantigens; Basement Membrane; Carrier Proteins; Chemical Phenomena; Chemistry; Collagen; Cytoskeletal Proteins; Dystonin; Glycoproteins; Heparitin Sulfate; Laminin; Membrane Glycoproteins; Membrane Proteins; Nerve Tissue Proteins; Non-Fibrillar Collagens; Osteonectin; Proteoglycans; Collagen Type XVII
PubMed: 3128422
DOI: 10.1016/s0174-173x(87)80042-0 -
Experimental Cell Research Jul 1994
Review
Topics: Animals; Autoantigens; Carrier Proteins; Cells, Cultured; Collagen; Cytoskeletal Proteins; Desmosomes; Dystonin; Extracellular Matrix; Humans; Intermediate Filament Proteins; Intermediate Filaments; Keratinocytes; Laminin; Models, Structural; Nerve Tissue Proteins; Non-Fibrillar Collagens; Pemphigoid, Bullous; Collagen Type XVII
PubMed: 8020577
DOI: 10.1006/excr.1994.1166 -
Current Biology : CB Dec 1996The quest for the function of BPAG1, a major hemidesmosomal protein of skin keratinocytes, has led to the discovery of a group of protein isoforms derived from the same... (Review)
Review
The quest for the function of BPAG1, a major hemidesmosomal protein of skin keratinocytes, has led to the discovery of a group of protein isoforms derived from the same genomic locus that are involved in organizing and integrating cytoskeletal networks in sensory neurons.
Topics: Animals; Autoantigens; Carrier Proteins; Collagen; Cytoskeletal Proteins; Cytoskeleton; Desmoplakins; Desmosomes; Dystonia Musculorum Deformans; Dystonin; Humans; Intermediate Filament Proteins; Keratins; Nerve Tissue Proteins; Neurons, Afferent; Non-Fibrillar Collagens; Plectin; Collagen Type XVII
PubMed: 8994813
DOI: 10.1016/s0960-9822(02)70772-0 -
Advances in Dermatology 2003BP180 is a key component of the epidermal anchoring complex and functions to maintain adherence of the epidermis to the basement membrane. Structural studies have... (Review)
Review
BP180 is a key component of the epidermal anchoring complex and functions to maintain adherence of the epidermis to the basement membrane. Structural studies have revealed that BP180 is a type II transmembrane protein with a long carboxy-terminal collagenous domain that projects into the extracellular region beneath the epidermal hemidesmosome. The collagenous domains have the characteristic tripeptide repeat, Gly-X-Y. A normal proteolytic processing event results in the shedding of the BP180 extracellular domain (LAD1) from the keratinocyte cell surface. The biologic relevance of this process is not yet known. The interactions of BP180 with other constituents of the anchoring complex have been extensively studied and underscore the importance of BP180 in the assembly and functioning of this cell-matrix adhesion structure. In addition to its role in maintaining the integrity of the dermal-epidermal junction, there is evidence that BP180 is involved in transmembrane signal transduction and in the regulation of keratinocyte differentiation. BP180 mutations are responsible for certain forms of JEB and a rare subform of epidermolysis bullosa simplex. In addition, 5 acquired blistering disorders (i.e. BP, HG, CP, LAD and LPP) are associated with an autoimmune response to BP180. In vivo and in vitro disease model systems have clearly established the pathogenic relevance of autoantibodies directed against specific sites on the BP180 extracellular domain. Molecular and cellular analyses of the autoimmune response in BP and HG have been unable to distinguish these 2 diseases, supporting the notion that HG can be considered a pregnancy-associated form of BP. In contrast, the anti-BP180 immune response of the other 3 disease--CP, LAD, and LPP--can be immunologically distinguished from BP and HG. The distinctions lie within the isotype and subclass of the autoantibodies, as well as in differences in their fine specificities or complement-fixing properties, or both. These differences are thought to account for the heterogeneous phenotypes observed in this family of autoimmune diseases.
Topics: Autoantigens; Carrier Proteins; Collagen; Cytoskeletal Proteins; Dystonin; Humans; Nerve Tissue Proteins; Non-Fibrillar Collagens; Pemphigoid, Bullous; Skin; Collagen Type XVII
PubMed: 14626817
DOI: No ID Found -
The Journal of Investigative Dermatology May 2021Bullous pemphigoid (BP) is an autoimmune blistering disease that targets the hemidesmosomal proteins BP180 and BP230/BPAG1e. Whereas the role of anti-BP180 antibodies...
Bullous pemphigoid (BP) is an autoimmune blistering disease that targets the hemidesmosomal proteins BP180 and BP230/BPAG1e. Whereas the role of anti-BP180 antibodies has been extensively characterized, the pathogenicity of anti-BPAG1e antibodies remains unclear. The purpose of this study is to elucidate the role of antibodies to BPAG1e in the experimental bullous pemphigoid models. We generated Bpag1 conditional knockout mice, where the knockout of Bpag1 is restricted to keratin 5-expressing epithelial cells. Bpag1 conditional knockout mice were immunized with the C-terminal portion of BPAG1e, and the splenocytes were injected into Rag2 mice intravenously. The recipient mice presented with erosion on the feet and tails. Microscopic examination showed subepidermal blisters and a linear deposition of IgG at the dermal-epidermal junction. To assess the potential role of trauma on BP development, we inflicted surface wounds on the dorsum of the Rag2 recipient mice after adoptive transfer. The wounded Rag2 mice had increased morbidity and severity of BP-like symptoms. Moreover, the depletion of B cells from splenocytes abolished a subepidermal blistering phenotype in vivo. These findings demonstrate that antibodies to BPAG1e might play a pathogenic role in causing subepidermal blistering, and external factors, including trauma, might be a trigger for BP development.
Topics: Animals; Autoantibodies; DNA-Binding Proteins; Disease Models, Animal; Dystonin; Immunization; Mice; Mice, Inbred C57BL; Pemphigoid, Bullous
PubMed: 33069726
DOI: 10.1016/j.jid.2020.08.031