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Marcel F. Jonkman - One of the best experts on this subject based on the ideXlab platform.
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the many faces of epidermolysis bullosa acquisita after serration pattern analysis by direct Immunofluorescence Microscopy
British Journal of Dermatology, 2011Co-Authors: J J A Buijsrogge, Gilles F H Diercks, Marcel F. JonkmanAbstract:Background The inflammatory variant of epidermolysis bullosa may mimic a form of pemphigoid. Objectives To estimate the frequency of epidermolysis bullosa acquisita (EBA) and bullous systemic lupus erythematosus (bSLE) among patients with subepidermal autoimmune bullous disease (sAIBD), and to correlate the isotype of in vivo antibody depositions to the clinical phenotype. Methods Patients with EBA or bSLE were systematically identified using serration pattern analysis by direct Immunofluorescence Microscopy in a prospective cohort of 364 patients with sAIBD. Correlation of the clinical phenotype to the isotype of the in vivo antibody depositions was investigated for 38 prospective and retrospective cases. Results The frequency of EBA or bSLE was 5.5% (n = 20), defined by the u-serration pattern, and reached only 1.9% (n = 7) when serological reactivity was the only criterion. The clinical phenotype of EBA was mechanobullous in 14 (37%) and inflammatory in 24 (63%) patients. Pure IgG-mediated cases (67%) were associated with the mechanobullous phenotype, whereas pure IgA-mediated cases (91%) were found more often in the inflammatory phenotype. Mucous membrane involvement was present in 22 (58%) patients, and neither correlated with IgG or IgA depositions, nor with a mechanobullous (64%) or inflammatory (54%) phenotype. Conclusions The frequency of EBA is about one in 18 among patients with sAIBD. The clinical phenotype in two of three cases is inflammatory, thus mimicking other sAIBDs, e. g. bullous pemphigoid, mucous membrane pemphigoid, or linear IgA disease. The yield of diagnosed EBA cases almost triples when serration pattern analysis is used by direct Immunofluorescence Microscopy on skin biopsy.
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Anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita: Differentiation by use of indirect Immunofluorescence Microscopy
Journal of the American Academy of Dermatology, 2003Co-Authors: Robert M. Vodegel, Kim B Yancey, Marcelus C.j.m. De Jong, Hendri H. Pas, Marcel F. JonkmanAbstract:Abstract Binding of autoantibodies to laminin 5 and type VII collagen causes anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita, respectively. Differentiation between these two dermal-binding autoimmune bullous dermatoses is not yet possible by indirect Immunofluorescence Microscopy. In this study we tested whether two recently described Immunofluorescence techniques, "knockout" skin substrate and fluorescent overlay antigen mapping, can differentiate between anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita. A total of 10 sera were tested: 4 with antilaminin 5, and 6 with antitype VII collagen autoantibodies, as characterized by either immunoblot or immunoprecipitation analysis. Differentiation between anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita was possible in all 10 sera by indirect Immunofluorescence using either knockout skin substrate or fluorescent overlay antigen mapping technique. (J Am Acad Dermatol 2003;48:542-7.)
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autoantibodies in anti p200 pemphigoid stain skin lacking laminin 5 and type vii collagen
British Journal of Dermatology, 2000Co-Authors: Detlef Zillikens, Marcel F. Jonkman, Takashi Hashimoto, A Ishiko, I Chimanovitch, E B BrockerAbstract:We report the case of a patient with a widespread bullous skin disease and linear deposits of IgG and C3 at the dermal-epidermal junction using direct Immunofluorescence Microscopy. Indirect Immunofluorescence analysis demonstrated circulating IgG autoantibodies that stained, like autoantibodies to laminin 5 and type VII collagen, the dermal side of 1 mol L-1 NaCl-split human skin. By immunoblotting dermal extracts, the patient's serum, like serum samples from two control patients, reacted with a 200-kDa protein. Using immunoelectron Microscopy, the serum labelled a component of the lower lamina lucida, but not the lamina densa/sublamina densa region, distinguishing this from the type VII collagen localization pattern. By Immunofluorescence Microscopy on skin sections from patients lacking either laminin 5 (Herlitz's epidermolysis bullosa) or type VII collagen (recessive dystrophic epidermolysis bullosa of Hallopeau-Siemens), the patient's serum retained reactivity with these test substrates. The patient's disease responded rapidly to the use of topical corticosteroids and lesions healed without scarring or milia formation. Our results provide strong evidence for the hypothesis that the 200 kDa autoantigen is different from laminin 5 and type VII collagen. For this new disease, we propose the designation 'anti-p200 pemphigoid'.
Kim B Yancey - One of the best experts on this subject based on the ideXlab platform.
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Anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita: Differentiation by use of indirect Immunofluorescence Microscopy
Journal of the American Academy of Dermatology, 2003Co-Authors: Robert M. Vodegel, Kim B Yancey, Marcelus C.j.m. De Jong, Hendri H. Pas, Marcel F. JonkmanAbstract:Abstract Binding of autoantibodies to laminin 5 and type VII collagen causes anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita, respectively. Differentiation between these two dermal-binding autoimmune bullous dermatoses is not yet possible by indirect Immunofluorescence Microscopy. In this study we tested whether two recently described Immunofluorescence techniques, "knockout" skin substrate and fluorescent overlay antigen mapping, can differentiate between anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita. A total of 10 sera were tested: 4 with antilaminin 5, and 6 with antitype VII collagen autoantibodies, as characterized by either immunoblot or immunoprecipitation analysis. Differentiation between anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita was possible in all 10 sera by indirect Immunofluorescence using either knockout skin substrate or fluorescent overlay antigen mapping technique. (J Am Acad Dermatol 2003;48:542-7.)
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anti epiligrin cicatricial pemphigoid a case associated with gastric carcinoma and features resembling epidermolysis bullosa acquisita
British Journal of Dermatology, 1998Co-Authors: Wataru Fujimoto, Kim B Yancey, Akemi Ishidayamamoto, Roger Hsu, Y Nagao, Hajime Iizuka, Jiro ArataAbstract:A 48-year-old woman with anti-epiligrin cicatricial pemphigoid (CP) who showed clinical features resembling epidermolysis bullosa acquisita was found to have adenocarcinoma of the stomach. Histological examination of lesional skin demonstrated a subepidermal blister. Direct Immunofluorescence Microscopy of perilesional skin revealed linear deposits of IgG and C3 at the basement membrane zone. The patient's serum contained IgG autoantibodies that bound to the dermal side of 1 mol/L NaCl-split normal human skin as determined by indirect Immunofluorescence Microscopy, and the lamina lucida as determined by indirect immunoelectron Microscopy. The patient's serum immunoprecipitated laminin-5 from extracts and media of biosynthetically radiolabelled human keratinocytes. Immunoblot studies showed that the patient's autoantibodies specifically bound the alpha3 subunit of this laminin isoform. Fragility of the skin and bullous lesions disappeared after total gastrectomy, but soon reappeared possibly in association with metastatic disease in a lymph node. The possibility that anti-epiligrin CP may develop paraneoplastically in some patients is discussed.
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reactivity of autoantibodies from patients with defined subepidermal bullous diseases against 1 mol l salt split skin specificity sensitivity and practical considerations
Journal of The American Academy of Dermatology, 1996Co-Authors: Zelmira Lazarova, Kim B YanceyAbstract:Abstract Background: Circulating IgG anti-basement membrane autoantibodies from patients with subepidermal bullous diseases can be categorized on the basis of their pattern of reactivity against 1 mol/L sodium chloride (NaCl)-split skin. Objective: The purpose of this study was to define by immunochemical techniques the specific antigen(s) targeted by IgG autoantibodies from a group of patients with subepidermal blistering diseases and then (1) prospectively determine which side(s) of 1 mol/L NaCl-split skin is (are) bound by these patients' autoantibodies, (2) compare the sensitivity of intact and 1 mol/L NaCl-split skin for the detection of these autoantibodies; and (3) devise a practical method to distinguish patients with antiepiligrin cicatricial pemphigoid from those with other subepidermal blistering diseases. Methods: Investigative techniques included direct and indirect Immunofluorescence Microscopy, immunoprecipitation studies, and immunoblotting. Results: These studies identified 14 patients whose sera immunoprecipitate bullous pemphigoid antigens 1, 2, or both. These patients' circulating IgG anti-basement membrane autoantibodies bind the epidermal ( n = 11), epidermal and dermal ( n = 2), or dermal ( n = 1) sides of 1 mol/L NaCl-split skin by indirect Immunofluorescence Microscopy. In contrast, IgG from all patients with autoantibodies directed against type VII collagen ( n = 5) or epiligrin ( n = 6) bind only the dermal side of 1 mol/L NaCl-split skin. In all but one patient in this series, 1 mol/L NaCl-split skin proved to be a more sensitive test substrate than intact human skin for detection of circulating IgG anti-basement membrane autoantibodies. Patients with antiepiligrin cicatricial pemphigoid were distinguished from other patients in that their circulating autoantibodies bound epidermal basement membrane in the skin of primates but not small mammals. Conclusion: NaCl-split skin (1 mol/L) of various species is a sensitive and practical indirect Immunofluorescence Microscopy test substrate for the evaluation of patients with IgG anti-basement membrane autoantibodies and evaluation of subepidermal bullous diseases.
Nigel Chaffey - One of the best experts on this subject based on the ideXlab platform.
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cortical microtubule involvement in bordered pit formation in secondary xylem vessel elements of aesculus hippocastanum l hippocastanaceae a correlative study using electron Microscopy and indirect Immunofluorescence Microscopy
Protoplasma, 1997Co-Authors: J R Barnett, Nigel Chaffey, Peter BarlowAbstract:A correlative study, using indirect Immunofluorescence Microscopy (IIF) of anti-α-tubulin stained sections and transmission electron Microscopy (TEM), gave details of the involvement of cortical microtubules (CMTs) in the development of bordered pits in secondary xylem vessel elements ofAesculus hippocastanum L. In addition, aspects of wall cytochemistry were studied during this process using the Thiery (PATAg) test, immunolocalization with the monoclonal antibodies JIM5 and JIM7, and a range of other cytochemical procedures. IIF showed that the alternately-arranged pits are pre-figured as perforations within a reticulum of randomly-oriented CMTs before any secondary wall thickening is obvious. Each incipient pit border is subsequently delimited by a circle of CMTs whose diameter decreases as deposition of secondary wall takes place around the perforation. These IIF observations are corroborated by a parallel TEM study. During the period of bordered pit formation, the secondary walls of the cell are lignifying. At maturity, however, the pit membrane is unlignified and continues to stain strongly with the monoclonal antibody JIM5, a marker of primary, “juvenile” wall. The results are discussed in terms of the relationship of the CMT cytoskeleton with development of bordered pits.
Enno Schmidt - One of the best experts on this subject based on the ideXlab platform.
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epidermolysis bullosa acquisita and anti p200 pemphigoid as major subepidermal autoimmune bullous diseases diagnosed by floor binding on indirect Immunofluorescence Microscopy using human salt split skin
Indian Journal of Dermatology Venereology and Leprology, 2017Co-Authors: Nupur Goyal, Raghavendra Rao, Sathish B Pai, Enno Schmidt, Shrutakirthi D Shenoi, Pramod Kumar, B S Bhogal, Detlef ZillikensAbstract:Background: Subepidermal autoimmune bullous diseases are a diverse group of diseases with overlapping clinical and immunopathological features. Indirect Immunofluorescence Microscopy on artificially split skin helps to classify these conditions into those with staining on the epidermal side of the split (“roof-binding”) and those with staining on the dermal side (“floor-binding”). Epidermolysis bullosa acquisita is the prototype of “floor-binding” subepidermal autoimmune bullous diseases. However, not all floor-binding sera are associated with epidermolysis bullosa acquisita. Aim: The aim of this study was to evaluate the clinical and immunological profile of patients with floor-binding subepidermal autoimmune bullous disease by indirect Immunofluorescence Microscopy and to identify the target antigens in them. Methods: Ten patients who showed a floor-binding pattern were studied with regard to their clinical and immunopathological characteristics. Target antigens were identified by modified indirect Immunofluorescence Microscopy using recessive dystrophic epidermolysis bullosa skin, enzyme linked immunosorbent assay, and immunoblotting. Results: Diagnosis of epidermolysis bullosa acquisita was confirmed in six patients. Three patients with an inflammatory subepidermal autoimmune bullous disease mimicking bullous pemphigoid reacted with a 200 kDa protein on immunoblotting with dermal extract, as is characteristic of anti-p200 pemphigoid. One serum showed both roof and floor binding, and reacted with the BP180 antigen. Limitation: We could not perform serration pattern analysis in our patients. Conclusion: In this study, we report three cases of anti-p200 pemphigoid from India. These cases, though indistinguishable clinically from bullous pemphigoid, revealed a floor-binding pattern on indirect Immunofluorescence using salt-split skin.
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Laboratory Diagnosis and Clinical Profile of Anti-p200 Pemphigoid
JAMA dermatology, 2016Co-Authors: Joost M. Meijer, Gilles F H Diercks, Enno SchmidtAbstract:Importance Anti-p200 pemphigoid is a rare subepidermal autoimmune blistering disease characterized by autoantibodies against a 200-kDa protein in the basement membrane zone. Anti-p200 pemphigoid is probably often misdiagnosed because of low availability of diagnostic assays and expertise and classified as bullous pemphigoid or epidermolysis bullosa acquisita. Objective To clinically characterize patients with anti-p200 pemphigoid, identified by using indirect Immunofluorescence Microscopy on skin substrates deficient in type VII collagen and laminin-332 (knockout analysis), to validate this technique by immunoblot with dermal extract, and to incorporate direct Immunofluorescence serration pattern analysis in the diagnostic algorithm. Design, Setting, and Participants This was a retrospective study performed from January 2014 to June 2015 with biobank patient materials and clinical data for the period 1998 to 2015 from the single national referral center on autoimmune bullous diseases. Patients were selected based on a dermal side binding on 1-mol/L salt (sodium chloride)-split human skin substrate by indirect Immunofluorescence Microscopy, not diagnosed epidermolysis bullosa acquisita or anti–laminin-332 mucous membrane pemphigoid. Main Outcomes and Measures Indirect Immunofluorescence Microscopy knockout analysis was performed and diagnosis of anti-p200 confirmed by immunoblot with dermal extract. Clinical, histological, and immunological findings were registered. Autoantibodies against laminin γ1 were determined by immunoblot. Results Twelve patients with anti-p200 pemphigoid (7 male and 5 female; mean age, 66.6 years) were identified using the indirect Immunofluorescence Microscopy knockout analysis. Direct Immunofluorescence Microscopy showed a linear n-serrated IgG deposition pattern along the basement membrane zone in 9 of 11 patients. The diagnosis was confirmed by immunoblot showing autoantibodies against 200-kDa protein in dermal extract in 12 of 12 patients. Autoantibodies against recombinant laminin γ1 were detected by immunoblot in 8 of 12 patients. Remarkable similarities were seen in clinical features with predominantly tense blisters on hands and feet, resembling dyshidrosiform pemphigoid. Mucosal involvement was seen in 6 (50%) of the patients. Conclusions and Relevance Predominance of blisters on hands and feet may be a clinical clue to the diagnosis of anti-p200 pemphigoid. Direct Immunofluorescence Microscopy serration pattern analysis and indirect Immunofluorescence Microscopy knockout analysis are valuable additional techniques to facilitate the diagnosis of anti-p200 pemphigoid.
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clinical features and practical diagnosis of bullous pemphigoid
Immunology and Allergy Clinics of North America, 2012Co-Authors: Enno Schmidt, Rocco Della Torre, Luca BorradoriAbstract:Bullous pemphigoid (BP) represents the most common autoimmune subepidermal blistering disease. BP typically affects the elderly and is associated with significant morbidity. It has usually a chronic course with spontaneous exacerbations. The cutaneous manifestations of BP can be extremely protean. While diagnosis of BP in the bullous stage is straightforward, in the non-bullous stage or in atypical variants of BP signs and symptoms are frequently non-specific with eg, only itchy excoriated, eczematous, papular and/or urticarial lesions that may persist for several weeks or months. Diagnosis of BP critically relies on immunopathologic examinations including direct Immunofluorescence Microscopy and detection of serum autoantibodies by indirect Immunofluorescence Microscopy or BP180-ELISA.
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premonitory epidermolysis bullosa acquisita mimicking eyelid dermatitis successful treatment with rituximab and protein a immunoapheresis
American Journal of Clinical Dermatology, 2010Co-Authors: Ilja Kubisch, Enno Schmidt, Philip Diessenbacher, Harald Gollnick, Martin LeverkusAbstract:We report a unique case of a 71-year-old female patient with epidermolysis bullosa acquisita. The patient initially presented with the clinical symptoms of bilateral eyelid dermatitis that occurred several months prior to the development of oral and pharyngeal erosions and blisters. While no contact allergy was found by patch testing, direct Immunofluorescence Microscopy demonstrated linear deposits of IgG at the basement membrane zone. By indirect Immunofluorescence Microscopy on human salt-split skin, IgG antibodies bound to the dermal side of the split. Immunoblot analysis showed predominant IgG4 reactivity of the patient's serum with the recombinant non-collagenous-1 domain of type VII collagen. Because of treatment resistance to systemic corticosteroids, dapsone, and colchicine, we initiated a combination treatment of protein A immunoapheresis and rituximab. With this treatment, complete remission was achieved within 4 months. Our case highlights that epidermolysis bullosa acquisita may initially mimic an eyelid dermatitis. Consequently, dermatologists should be aware of this rare differential diagnosis of eyelid dermatitis where a contact allergy or atopic dermatitis is absent.
Peter Barlow - One of the best experts on this subject based on the ideXlab platform.
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cortical microtubule involvement in bordered pit formation in secondary xylem vessel elements of aesculus hippocastanum l hippocastanaceae a correlative study using electron Microscopy and indirect Immunofluorescence Microscopy
Protoplasma, 1997Co-Authors: J R Barnett, Nigel Chaffey, Peter BarlowAbstract:A correlative study, using indirect Immunofluorescence Microscopy (IIF) of anti-α-tubulin stained sections and transmission electron Microscopy (TEM), gave details of the involvement of cortical microtubules (CMTs) in the development of bordered pits in secondary xylem vessel elements ofAesculus hippocastanum L. In addition, aspects of wall cytochemistry were studied during this process using the Thiery (PATAg) test, immunolocalization with the monoclonal antibodies JIM5 and JIM7, and a range of other cytochemical procedures. IIF showed that the alternately-arranged pits are pre-figured as perforations within a reticulum of randomly-oriented CMTs before any secondary wall thickening is obvious. Each incipient pit border is subsequently delimited by a circle of CMTs whose diameter decreases as deposition of secondary wall takes place around the perforation. These IIF observations are corroborated by a parallel TEM study. During the period of bordered pit formation, the secondary walls of the cell are lignifying. At maturity, however, the pit membrane is unlignified and continues to stain strongly with the monoclonal antibody JIM5, a marker of primary, “juvenile” wall. The results are discussed in terms of the relationship of the CMT cytoskeleton with development of bordered pits.
