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Takashi Hashimoto - One of the best experts on this subject based on the ideXlab platform.
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pathogenic activation and therapeutic blockage of fc alpha receptor expressing polymorphonuclear leukocytes in IgA Pemphigus
Journal of Investigative Dermatology, 2021Co-Authors: Shirin Emtenani, Khalaf Kridin, Takashi Hashimoto, Saeedeh Ghorbanalipoor, Sarah Mayerhain, L Komorowski, Christian Probst, Hendri H Pas, Kaja Mecinskajundzill, Rafal CzajkowskiAbstract:Pathomechanisms in IgA Pemphigus are assumed to rely on Fc-dependent cellular activation by antigen-specific IgA autoantibodies, however, models for disease and more detailed pathophysiologic data are lacking. We here aimed to establish in vitro models of disease for IgA Pemphigus, allowing to study effects of the interaction of anti-keratinocyte IgA with cell-surface Fc alpha receptors. Employing multiple in vitro assays, such as a skin cryosection assay and a human skin organ culture model, we here present mechanistic data for the pathogenesis of IgA Pemphigus, mediated by anti-desmoglein 3 IgA autoantibodies. Our results reveal that this disease is dependent on Fc alpha receptor-mediated activation of leukocytes in the epidermis. Importantly, this cell-dependent pathology can be dose-dependently abrogated by peptide-mediated inhibition of Fc alpha receptor:IgA interaction, as confirmed in an additional model for IgA-dependent disease, i.e., IgA vasculitis. These data suggest that IgA Pemphigus can be modeled in vitro, and IgA Pemphigus and IgA vasculitis are Fc alpha receptor-dependent disease entities that can be specifically targeted in these experimental systems.
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clinical and immunological studies of 49 cases of various types of intercellular IgA dermatosis and 13 cases of classical subcorneal pustular dermatosis examined at kurume university
British Journal of Dermatology, 2017Co-Authors: Takashi Hashimoto, Kwesi Teye, Norito IshiiAbstract:Background Intercellular IgA dermatosis (IAD) is a subset of autoimmune bullous disease exclusively with IgA antikeratinocyte cell-surface antibodies. The classification and pathogenesis of this condition are still obscure. Objectives To classify IAD and study its pathogenesis. Methods From our cohort of 5402 cases of autoimmune bullous disease, we selected 49 cases of various types of intercellular IgA dermatosis (IAD) and 13 cases of classical subcorneal pustular dermatosis (SPD), for which sera and information were available. We studied these cases clinically and immunologically. Results There were 17 SPD-type IAD, 12 intraepidermal neutrophilic IgA dermatosis (IEN)-type IAD, two IgA-Pemphigus vegetans, four IgA-Pemphigus foliaceus, six IgA-Pemphigus vulgaris and eight unclassified IAD cases. There was no sex predominance, and the average age at disease onset was 45·9 years. Clinically, bullous and pustular skin lesions developed on various sites, particularly intertriginous areas. Histopathology showed intraepidermal blisters or pustules at the upper epidermis in the SPD-type and at the midepidermis in the IEN-type. Immunological studies revealed that direct immunofluorescence, indirect immunofluorescence of normal human skin and enzyme-linked immunosorbent assays (ELISAs) of recombinant proteins of desmogleins and desmocollins frequently showed positive results, although no antigens were detected in many cases. All cases of classical SPD, which showed no positive immunological results, were indistinguishable clinically and histopathologically from SPD-type IAD. Conclusions The present study of the largest cohort of cases of IAD showed that the major subtypes are SPD and IEN, and that the combination of indirect immunofluorescence and ELISAs of desmogleins and desmocollins, in addition to direct immunofluorescence, was useful for the diagnosis of IAD and its subtypes.
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detection of igg antibodies to desmoglein 3 and desmocollins 2 and 3 in mucosal dominant type Pemphigus vulgaris with severe pharyngalgia and hyperemia of the bulbar conjunctiva
European Journal of Dermatology, 2015Co-Authors: Teruhiko Makino, Norito Ishii, Takashi Hashimoto, Hiroshi Hara, Megumi Mizawa, Yuri Seki, Masao Hayashi, Tadamichi ShimizuAbstract:Pemphigus vulgaris (PV) is an autoimmune blistering skin disease where desmogleins (Dsgs), desmosomal cadherin-type cell adhesion molecules, are major autoantigens [1]. Desmocollins (Dscs) are another group of desmosomal cadherins [2]. Dsc1 was previously reported to be an autoantigen in subcorneal pustular dermatosis-type IgA Pemphigus [3]. In addition, anti-Dsc antibodies also exist in patients with paraneoplastic Pemphigus (PNP) or atypical Pemphigus [4, 5]. We report here a patient with mucosal [...]
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Epidermal polymeric immunoglobulin receptors: leads from intraepidermal neutrophilic IgA dermatosis-type IgA Pemphigus
Experimental dermatology, 2015Co-Authors: Atsunari Tsuchisaka, Norito Ishii, Takahiro Hamada, Hiroshi Koga, Kwesi Teye, Daisuke Tsuruta, Chika Ohata, Ryosuke Sogame, Minao Furumura, Takashi HashimotoAbstract:In this study, we attempted to identify unknown autoantigen for intraepidermal neutrophilic IgA dermatosis-type IgA Pemphigus by novel IgA-specific immunoprecipitation. Mass-spectrometry study identified polymeric immunoglobulin receptor (PIGR) as the candidate protein, and we confirmed that PIGR expressed in both epidermis and cultured keratinocytes. Eukaryotic recombinant protein of PIGR expressed in COS7 cells was reacted with both patient and normal sera, indicating that PIGR binds physiologically to IgA. To detect antigen-specific binding by IgA autoantibodies, we performed several experiments using deglycosylated PIGR and F(ab)2 fragments from patient sera. However, these analyses suggested that patient IgA bound physiologically, but not immunologically, to PIGR. Nevertheless, our study provided two important insights. Newly developed IgA-immunoprecipitation system should be a useful tool in the future study of identification of antigens for IgA autoantibodies. Detection of epidermal PIGR in this study confirmed previous results and indicated possible immunological role of PIGR in epidermis.
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igg IgA Pemphigus recognizing desmogleins 1 and 3 in a patient with sjogren s syndrome
European Journal of Dermatology, 2014Co-Authors: Aiko Furuya, Norito Ishii, Takashi Hashimoto, Eishi Takahashi, Takahiro SatohAbstract:A 66-year-old Japanese female presented with a one-month history of pustular lesions on the trunk and perineal regions. Physical examination revealed a number of varying-sized pustules rimmed by erythema (Fig. 1A). Some lesions showed flaccid pustules and/or blisters at the periphery and yellow crusts in the center of the erythematous plaque (figure 1B). Oral mucosa showed extensive erosions, which appeared approximately two weeks before the admission to our hospital. Her past history was unremarkable, [...]
Masayuki Amagai - One of the best experts on this subject based on the ideXlab platform.
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IgA Pemphigus reacting exclusively to desmoglein 3
European Journal of Dermatology, 2010Co-Authors: Mami Tajima, Takashi Hashimoto, Yoshihiko Mitsuhashi, Ryokichi Irisawa, Masayuki Amagai, Ryoji TsuboiAbstract:IgA Pemphigus is a rare disease which is classified into two major types: subcorneal pustular dermatosis (SPD) and intraepidermal neutrophilic IgA dermatosis (IEN). The autoantigen of the SPD type was identified as desmocollin1 (Dsc1), while the antigen of the IEN type is still unknown. We report a case of IgA Pemphigus possessing antibodies exclusively against desmoglein3 (Dsg3). The patient also had ulcerative colitis, clinically severe oral mucosal lesions with pustule formation, and the "sunflower-like" pustular skin lesions. Histopathology showed intraepidermal bullae with numerous neutrophils. Direct immunofluorescence showed IgA deposits in the keratinocyte cell surfaces only in the lower epidermis. Indirect immunofluorescence using normal human skin as a substrate showed circulating IgA anti-cell surface autoantibodies at a titer of 1:160. cDNA transfection test for Dsc1-3 was negative. IgA antibodies to Dsg3, but not Dsg1, were detected with IgA-ELISA for Dsgs. Addition of diaphenylsulfone (DDS) to prednisolone and cyclosporine A significantly improved the oral and skin lesions. There have been rare cases showing IgA antibodies to either Dsg1 or Dsg3. These cases can be designated as IgA Pemphigus foliaceus or IgA Pemphigus vulgaris, respectively, according to the autoantigens. We report here a case of IgA Pemphigus vulgaris complicated by ulcerative colitis.
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IgA igg Pemphigus a new atypical subset of Pemphigus
Acta Dermato-venereologica, 2006Co-Authors: Cezary Kowalewski, Takashi Hashimoto, Masayuki Amagai, Stefania Jablonska, Wojciech Mackiewicz, Katarzyna WozniakAbstract:The classical types of Pemphigus are well characterized clinically, histologically and immunologically. The tar-get antigens were found to be desmoglein 1 (Dsg1) for Pemphigus foliaceus and Dsg3 for Pemphigus vulgaris (1). However, there are atypical cases which could not be classified into either of these types. At present two major atypical forms are recognized: Pemphigus herpetiformis (PH) (2, 3) and IgA Pemphigus with its subsets: subcor-neal pustular dermatosis (SPD) (4–6) and intraepidermal neutrophilic IgA dermatosis (IEN) (7–9).The clinical pattern of PH and IgA Pemphigus may to varying degrees resemble dermatitis herpetiformis (eryt-hematous, herpetiform eruption, often in annular arrange-ment). The histological pattern is not characteristic since subcorneal acantholysis may be slight or absent (10). Since the clinical and histological differentiation of PH and IgA Pemphigus might be difficult or even not possible, the diagnosis is based on immunological findings: IgG antibodies in PH (3) and IgA antibodies in IgA Pemphigus (6, 8, 11). The most interesting problem is coexistence in some cases of both IgA and IgG antibodies, referred to as IgA/IgG Pemphigus (12). Since this subset of Pemphigus is still not known we present under this name a case with clinical and histological features of PH and immunological pattern of IgA Pemphigus of IEN type and reactivity of both IgG and IgA antibodies exclusively to Dsg1.CASE REPORT
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igg IgA Pemphigus with igg and IgA antidesmoglein 1 antibodies detected by enzyme linked immunosorbent assay
British Journal of Dermatology, 2002Co-Authors: Naoki Oiso, Takashi Hashimoto, Y. Nagata, Masayuki Amagai, Ayako Komai, C Yamashita, K Yoshioka, Masamitsu IshiiAbstract:Pemphigus is an autoimmune mucocutaneous bullous disease characterized by autoantibodies against the cell surfaces of epidermal keratinocytes. Six cases with deposition of both IgG and IgA on keratinocyte cell surfaces have been reported in the recent literature. We provisionally termed these cases IgG/IgA Pemphigus. We describe a 42-year-old Japanese woman with clinical and histopathological features resembling herpetiform Pemphigus who demonstrated in vivo bound and circulating anticell surface autoantibodies of both IgG and IgA classes on immunofluorescence examination. Enzyme-linked immunosorbent assay using baculovirus-expressed recombinant desmoglein (Dsg) 1 and Dsg 3 showed that both IgG and IgA antibodies reacted with Dsg1. The reactivity was completely adsorbed with preincubation of serum with Dsg1 baculoprotein, further confirming the exclusive reactivity of both IgG and IgA antibodies with Dsg1. This is the second case of IgG/IgA Pemphigus in which the human target antigens for both IgG and IgA antibodies have been unequivocally identified. This study provides further evidence that IgG/IgA Pemphigus is a distinct disease entity.
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detection of IgA autoantibodies to desmogleins by an enzyme linked immunosorbent assay the presence of new minor subtypes of IgA Pemphigus
Archives of Dermatology, 2001Co-Authors: Takashi Hashimoto, Ayako Komai, Yuko Futei, Takeji Nishikawa, Masayuki AmagaiAbstract:Objective To examine the frequency of antidesmoglein 1 (Dsg1) and antidesmoglein 3 (Dsg3) IgA autoantibodies in IgA Pemphigus. Design We developed an enzyme-linked immunosorbent assay against recombinant Dsg1 and Dsg3 to detect IgA autoantibodies. Patients Twenty-two patients with IgA Pemphigus were studied. Among them, 10 patients had subcorneal pustular dermatosis type, 9 patients had intraepidermal neutrophilic IgA dermatosis type, and 3 patients had Pemphigus foliaceus–like clinical features. Results Of the 22 cases of IgA Pemphigus, 3 cases were positive for anti-Dsg1 IgA antibodies and only 1 case was positive for anti-Dsg3 IgA antibodies. In those 4 cases, there were no IgA autoantibodies against other components of the keratinocyte cell surfaces because preincubation with the respective recombinant desmogleins removed the immunoreactivity on immunofluorescence. All 10 patients with subcorneal pustular dermatosis type IgA Pemphigus were positive against desmocollin 1 expressed on COS-7 cells. No target antigen was detected in the other 8 cases. Conclusions Desmogleins were recognized by IgA antibodies of a few patients with IgA Pemphigus. Considering that subcorneal pustular dermatosis type IgA Pemphigus recognizes desmocollin 1, autoimmune targets of IgA Pemphigus are more heterogeneous than previously considered.
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rapid response of IgA Pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin
Journal of The American Academy of Dermatology, 2000Co-Authors: M Kroiss, Detlef Zillikens, Takashi Hashimoto, Masayuki Amagai, Claus GrussAbstract:Diagnosing IgA Pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA dermatosis type and subcorneal pustular dermatosis type, is important because treatment of IgA Pemphigus has to be different from treatment of other blistering autoimmune dermatoses. We present a patient with subcorneal pustular dermatosis type of IgA Pemphigus who rapidly responded to systemic treatment with isotretinoin. Specific diagnosis was established by detecting IgA serum activity to desmocollin 1 by indirect immunofluorescence microscopy on unfixed COS7 cells transfected with desmocollin 1. No IgA or IgG serum reactivity was found to recombinant forms of desmogleins 1 and 3 by an antigen-specific enzyme-linked immunosorbent assay. The disease was not effectively controlled by conventional therapeutic regimens. Systemic treatment with isotretinoin 20 mg daily led to complete clearance of skin lesions within 3 weeks. Assaying IgA serum reactivity to desmocollin 1, desmoglein 1, and desmoglein 3 as a valuable method for establishing the diagnosis and differentiating the 2 subtypes of IgA Pemphigus. Isotretinoin was an effective drug in the treatment of subcorneal pustular dermatosis type of IgA Pemphigus in this patient.
Detlef Zillikens - One of the best experts on this subject based on the ideXlab platform.
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subcorneal pustular dermatosis type IgA Pemphigus with autoantibodies to desmocollins 1 2 and 3
Archives of Dermatology, 2009Co-Authors: Imke Duker, Christian Rose, T Hashimoto, Detlef Zillikens, Jörg Schaller, Johannes KunzeAbstract:Background IgA Pemphigus is a rare neutrophilic acantholytic autoimmune disease that is characterized by IgA deposits on keratinocyte cell surfaces. Clinically and histologically, IgA Pemphigus is divided into 2 major subtypes: subcorneal pustular dermatosis and intraepidermal neutrophilic IgA dermatosis. We report the first case of subcorneal pustular dermatosis–type IgA Pemphigus that showed reactivity to all 3 isoforms of the desmocollin family by indirect immunofluorescence microscopy of COS7 cells transfected with desmocollin 1, 2, or 3. Observations We describe a 94-year-old woman with IgA Pemphigus with a unique immunopathologic pattern. Direct immunofluorescence microscopy revealed IgA deposits throughout the entire epidermis, with stronger staining in the upper epidermis. The autoantibodies from this patient did not show IgA or IgG reactivity with desmogleins via immunoblotting or enzyme-linked immunosorbent assay. By indirect immunofluorescence by the use of COS7 cells transfected with desmocollin 1, 2, or 3, IgA autoantibodies in a serum sample from our patient clearly reacted with all of them. Conclusions The pathophysiology and autoantigen profile of bullous autoimmune diseases, especially Pemphigus and its subforms, are more complex than previously believed. Because Pemphigus seems to be a heterogeneous disorder, further studies are needed to evaluate the complexity of the disease.
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IgA Pemphigus occurrence of anti desmocollin 1 and anti desmoglein 1 antibody reactivity in an individual patient
Journal Der Deutschen Dermatologischen Gesellschaft, 2006Co-Authors: Tamara Kopp, Detlef Zillikens, Cassian Sitaru, Friederike Pieczkowski, Achim Schneeberger, Dagmar Födinger, Georg Stingl, Franz KarlhoferAbstract:Summary Background: IgA Pemphigus is a rare pustular autoimmune disease with exclusive IgA anti-keratinocyte cell surface antibody reactivity. Two subtypes have been discerned: in the subcorneal pustular dermatosis type, desmocollin 1 has been identified as a targeted autoantigen, while in few cases of the intraepidermal neutrophilic type, IgA anti-desmoglein 1 or IgA anti-desmoglein 3 reactivity has been demonstrated. Patients and Methods: A 48-year-old white male presented with generalized large confluent pustules. Skin pathology was assessed by histology and direct immunofluorescence analysis. IgG/IgA autoantibodies against desmoglein 1/3 and desmocollin 1 were measured by ELISA and indirect immunofluorescence using desmocollin 1 cDNA-transfected COS7 cells, respectively. Results: Histopathology revealed subcorneal pustules and direct immunofluorescence microscopy exclusively showed in vivo bound IgA with an intercellular pattern in the epidermis. Desmocollin 1 was identified as a target of IgA autoantibodies by indirect immunofluorescence microscopy utilizing desmocollin 1 cDNA-transfected COS7 cells. In addition, IgA anti-desmoglein 1 reactivity was demonstrated by ELISA. Neither IgA anti-desmoglein 3 nor IgG anti-desmoglein 1/3 autoantibodies were present. Conclusions: Both desmocollin 1 and desmoglein 1 were autoantigens in this patient with IgA Pemphigus and a distinct clinical presentation. To our knowledge, this is the first IgA Pemphigus case with dual autoantibody reactivity. Zusammenfassung Hintergrund: Der IgA Pemphigus ist eine seltene pustulose Autoimmunerkrankung, die sich durch IgA Antikorperreaktivitat gegen Oberflachenmolekule an Keratinozyten auszeichnet.Man unterscheidet zwei Subtypen: den subkornealpustulosen Dermatose-Typ, bei dem Desmocollin 1 das Autoantigen ist, und den intraepidermal-neutrophilen Typ, bei dem in einigen Fallen IgA-anti-Desmoglein-1- oder IgA-anti-Desmoglein-3-Reaktivitat gefunden wurde. Patienten und Methodik: Wir berichten uber einen 48-jahrigen kaukasischen Mann, der sich mit generalisiert auftretenden konfluierenden Pusteln prasentierte. Die zugrunde liegende kutane Immunpathologie wurde mittels histologischer Untersuchung und direkter Immunfluoreszenz analysiert.IgG/IgA-Autoantikorper gegen Desmoglein 1/3 und gegen Desmocollin 1 wurden mittels ELISA und indirekter Immunofluoreszenz an Desmocollin-1-cDNA-transfizierten COS7-Zellen bestimmt. Ergebnisse: Histopathologisch zeigten sich subkorneale Pusteln. In der direkten Immunofluoreszenz waren ausschlieslich in vivo gebundene IgA-Autoantikorper mit einem Interzellularmuster in der Epidermis nachweisbar. Desmocollin 1 wurde mittels indirekter Immunofluoreszenz an Desmocollin-1-cDNA-transfizierten COS7-Zellen als Zielstruktur der IgA-Autoantikorper identifiziert. Zusatzlich konnte IgA-anti-Desmoglein-1-Reaktivitat im ELISA nachgewiesen werden. Weder IgA-anti-Desmoglein-3- noch IgG-anti-Desmoglein-1/3-Autoantikorper waren detektierbar. Schlussfolgerungen: Zusammenfassend identifizieren diese Daten Desmocollin 1 und Desmoglein 1 als Autoantigene bei diesem Patienten. Soweit uns bekannt ist, ist dies der erste Fall eines IgA-Pemphigus mit dualer Autoantikorper-Reaktivitat.
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IgA Pemphigus vorkommen von anti desmocollin 1 und anti desmoglein 1 antikorpern bei einem patienten
Journal Der Deutschen Dermatologischen Gesellschaft, 2006Co-Authors: Tamara Kopp, Detlef Zillikens, Cassian Sitaru, Friederike Pieczkowski, Achim Schneeberger, Dagmar Födinger, Georg Stingl, Franz KarlhoferAbstract:Background: IgA Pemphigus is a rare pustular autoimmune disease with exclusive IgA anti-keratinocyte cell surface antibody reactivity. Two subtypes have been discerned: in the subcorneal pustular dermatosis type, desmocollin 1 has been identified as a targeted autoantigen, while in few cases of the intraepidermal neutrophilic type, IgA anti-desmoglein 1 or IgA anti-desmoglein 3 reactivity has been demonstrated. Patients and Methods: A 48-year-old white male presented with generalized large confluent pustules. Skin pathology was assessed by histology and direct immunofluorescence analysis. IgG/lgA autoantibodies against desmoglein 1/3 and desmocollin 1 were measured by ELISA and indirect immunofluorescence using desmocollin 1 cDNA-transfected COS7 cells, respectively. Results: Histopathology revealed subcorneal pustules and direct immunofluorescence microscopy exclusively showed in vivo bound IgA with an intercellular pattern in the epidermis. Desmocollin 1 was identified as a target of IgA autoantibodies by indirect immunofluorescence microscopy utilizing desmocollin 1 cDNA-transfected COS7 cells. In addition, IgA anti-desmoglein 1 reactivity was demonstrated by ELISA. Neither IgA anti-desmoglein 3 nor IgG anti-desmoglein 1/3 autoantibodies were present. Conclusions: Both desmocollin 1 and desmoglein 1 were autoantigens in this patient with IgA Pemphigus and a distinct clinical presentation. To our knowledge, this is the first IgA Pemphigus case with dual autoantibody reactivity.
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erfolgreiche therapie des IgA Pemphigus vom subkorneal pustulosen typ
Hautarzt, 2005Co-Authors: Ulrike Raap, Detlef Zillikens, Takashi Hashimoto, B Volker, H Petering, Alexander Kapp, T WerfelAbstract:Der IgA-Pemphigus ist eine seltene Erkrankung aus der Gruppe der bullosen Autoimmundermatosen, der durch Autoantikorper der Klasse IgA, die gegen Zielstrukturen (Cadherine) der Epidermis gerichtet sind, charakterisiert wird. Das Manifestationsalter des IgA-Pemphigus betrifft das 50. Lebensjahr. Histopathologische Hauptmerkmale des IgA-Pemphigus sind die Akantholyse mit subkornealer oder intraepidermaler Pustulation und teilweise epidermaler Infiltration neutrophiler Granulozyten. Charakteristisch fur das klinische Erscheinungsbild sind erythematose Papeln, Pusteln, Krusten, Schuppen und Erosionen, die insbesondere am Rumpf, an den Axillen, Leisten und proximalen Extremitaten auftreten. Das Mittel der Wahl zur Therapie des IgA-Pemphigus ist Diaminodiphenylsulfon. In der vorliegenden Kasuistik berichten wir uber einen Patienten, der an einem IgA-Pemphigus erkrankt war und erfolgreich mit Diaminodiphenylsulfon behandelt wurde.
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rapid response of IgA Pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin
Journal of The American Academy of Dermatology, 2000Co-Authors: Claudia Gruss, M Kroiss, Markus Landthaler, T. Vogt, T Hashimoto, Detlef Zillikens, W StolzAbstract:Abstract Diagnosing IgA Pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA dermatosis type and subcorneal pustular dermatosis type, is important because treatment of IgA Pemphigus has to be different from treatment of other blistering autoimmune dermatoses. We present a patient with subcorneal pustular dermatosis type of IgA Pemphigus who rapidly responded to systemic treatment with isotretinoin. Specific diagnosis was established by detecting IgA serum activity to desmocollin 1 by indirect immunofluorescence microscopy on unfixed COS7 cells transfected with desmocollin 1. No IgA or IgG serum reactivity was found to recombinant forms of desmogleins 1 and 3 by an antigen-specific enzyme-linked immunosorbent assay. The disease was not effectively controlled by conventional therapeutic regimens. Systemic treatment with isotretinoin 20 mg daily led to complete clearance of skin lesions within 3 weeks. Assaying IgA serum reactivity to desmocollin 1, desmoglein 1, and desmoglein 3 as a valuable method for establishing the diagnosis and differentiating the 2 subtypes of IgA Pemphigus. Isotretinoin was an effective drug in the treatment of subcorneal pustular dermatosis type of IgA Pemphigus in this patient. (J Am Acad Dermatol 2000;43:923-6.)
M Kroiss - One of the best experts on this subject based on the ideXlab platform.
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rapid response of IgA Pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin
Journal of The American Academy of Dermatology, 2000Co-Authors: Claudia Gruss, M Kroiss, Markus Landthaler, T. Vogt, T Hashimoto, Detlef Zillikens, W StolzAbstract:Abstract Diagnosing IgA Pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA dermatosis type and subcorneal pustular dermatosis type, is important because treatment of IgA Pemphigus has to be different from treatment of other blistering autoimmune dermatoses. We present a patient with subcorneal pustular dermatosis type of IgA Pemphigus who rapidly responded to systemic treatment with isotretinoin. Specific diagnosis was established by detecting IgA serum activity to desmocollin 1 by indirect immunofluorescence microscopy on unfixed COS7 cells transfected with desmocollin 1. No IgA or IgG serum reactivity was found to recombinant forms of desmogleins 1 and 3 by an antigen-specific enzyme-linked immunosorbent assay. The disease was not effectively controlled by conventional therapeutic regimens. Systemic treatment with isotretinoin 20 mg daily led to complete clearance of skin lesions within 3 weeks. Assaying IgA serum reactivity to desmocollin 1, desmoglein 1, and desmoglein 3 as a valuable method for establishing the diagnosis and differentiating the 2 subtypes of IgA Pemphigus. Isotretinoin was an effective drug in the treatment of subcorneal pustular dermatosis type of IgA Pemphigus in this patient. (J Am Acad Dermatol 2000;43:923-6.)
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rapid response of IgA Pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin
Journal of The American Academy of Dermatology, 2000Co-Authors: M Kroiss, Detlef Zillikens, Takashi Hashimoto, Masayuki Amagai, Claus GrussAbstract:Diagnosing IgA Pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA dermatosis type and subcorneal pustular dermatosis type, is important because treatment of IgA Pemphigus has to be different from treatment of other blistering autoimmune dermatoses. We present a patient with subcorneal pustular dermatosis type of IgA Pemphigus who rapidly responded to systemic treatment with isotretinoin. Specific diagnosis was established by detecting IgA serum activity to desmocollin 1 by indirect immunofluorescence microscopy on unfixed COS7 cells transfected with desmocollin 1. No IgA or IgG serum reactivity was found to recombinant forms of desmogleins 1 and 3 by an antigen-specific enzyme-linked immunosorbent assay. The disease was not effectively controlled by conventional therapeutic regimens. Systemic treatment with isotretinoin 20 mg daily led to complete clearance of skin lesions within 3 weeks. Assaying IgA serum reactivity to desmocollin 1, desmoglein 1, and desmoglein 3 as a valuable method for establishing the diagnosis and differentiating the 2 subtypes of IgA Pemphigus. Isotretinoin was an effective drug in the treatment of subcorneal pustular dermatosis type of IgA Pemphigus in this patient.
Norito Ishii - One of the best experts on this subject based on the ideXlab platform.
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clinical and immunological studies of 49 cases of various types of intercellular IgA dermatosis and 13 cases of classical subcorneal pustular dermatosis examined at kurume university
British Journal of Dermatology, 2017Co-Authors: Takashi Hashimoto, Kwesi Teye, Norito IshiiAbstract:Background Intercellular IgA dermatosis (IAD) is a subset of autoimmune bullous disease exclusively with IgA antikeratinocyte cell-surface antibodies. The classification and pathogenesis of this condition are still obscure. Objectives To classify IAD and study its pathogenesis. Methods From our cohort of 5402 cases of autoimmune bullous disease, we selected 49 cases of various types of intercellular IgA dermatosis (IAD) and 13 cases of classical subcorneal pustular dermatosis (SPD), for which sera and information were available. We studied these cases clinically and immunologically. Results There were 17 SPD-type IAD, 12 intraepidermal neutrophilic IgA dermatosis (IEN)-type IAD, two IgA-Pemphigus vegetans, four IgA-Pemphigus foliaceus, six IgA-Pemphigus vulgaris and eight unclassified IAD cases. There was no sex predominance, and the average age at disease onset was 45·9 years. Clinically, bullous and pustular skin lesions developed on various sites, particularly intertriginous areas. Histopathology showed intraepidermal blisters or pustules at the upper epidermis in the SPD-type and at the midepidermis in the IEN-type. Immunological studies revealed that direct immunofluorescence, indirect immunofluorescence of normal human skin and enzyme-linked immunosorbent assays (ELISAs) of recombinant proteins of desmogleins and desmocollins frequently showed positive results, although no antigens were detected in many cases. All cases of classical SPD, which showed no positive immunological results, were indistinguishable clinically and histopathologically from SPD-type IAD. Conclusions The present study of the largest cohort of cases of IAD showed that the major subtypes are SPD and IEN, and that the combination of indirect immunofluorescence and ELISAs of desmogleins and desmocollins, in addition to direct immunofluorescence, was useful for the diagnosis of IAD and its subtypes.
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detection of igg antibodies to desmoglein 3 and desmocollins 2 and 3 in mucosal dominant type Pemphigus vulgaris with severe pharyngalgia and hyperemia of the bulbar conjunctiva
European Journal of Dermatology, 2015Co-Authors: Teruhiko Makino, Norito Ishii, Takashi Hashimoto, Hiroshi Hara, Megumi Mizawa, Yuri Seki, Masao Hayashi, Tadamichi ShimizuAbstract:Pemphigus vulgaris (PV) is an autoimmune blistering skin disease where desmogleins (Dsgs), desmosomal cadherin-type cell adhesion molecules, are major autoantigens [1]. Desmocollins (Dscs) are another group of desmosomal cadherins [2]. Dsc1 was previously reported to be an autoantigen in subcorneal pustular dermatosis-type IgA Pemphigus [3]. In addition, anti-Dsc antibodies also exist in patients with paraneoplastic Pemphigus (PNP) or atypical Pemphigus [4, 5]. We report here a patient with mucosal [...]
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Epidermal polymeric immunoglobulin receptors: leads from intraepidermal neutrophilic IgA dermatosis-type IgA Pemphigus
Experimental dermatology, 2015Co-Authors: Atsunari Tsuchisaka, Norito Ishii, Takahiro Hamada, Hiroshi Koga, Kwesi Teye, Daisuke Tsuruta, Chika Ohata, Ryosuke Sogame, Minao Furumura, Takashi HashimotoAbstract:In this study, we attempted to identify unknown autoantigen for intraepidermal neutrophilic IgA dermatosis-type IgA Pemphigus by novel IgA-specific immunoprecipitation. Mass-spectrometry study identified polymeric immunoglobulin receptor (PIGR) as the candidate protein, and we confirmed that PIGR expressed in both epidermis and cultured keratinocytes. Eukaryotic recombinant protein of PIGR expressed in COS7 cells was reacted with both patient and normal sera, indicating that PIGR binds physiologically to IgA. To detect antigen-specific binding by IgA autoantibodies, we performed several experiments using deglycosylated PIGR and F(ab)2 fragments from patient sera. However, these analyses suggested that patient IgA bound physiologically, but not immunologically, to PIGR. Nevertheless, our study provided two important insights. Newly developed IgA-immunoprecipitation system should be a useful tool in the future study of identification of antigens for IgA autoantibodies. Detection of epidermal PIGR in this study confirmed previous results and indicated possible immunological role of PIGR in epidermis.
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igg IgA Pemphigus recognizing desmogleins 1 and 3 in a patient with sjogren s syndrome
European Journal of Dermatology, 2014Co-Authors: Aiko Furuya, Norito Ishii, Takashi Hashimoto, Eishi Takahashi, Takahiro SatohAbstract:A 66-year-old Japanese female presented with a one-month history of pustular lesions on the trunk and perineal regions. Physical examination revealed a number of varying-sized pustules rimmed by erythema (Fig. 1A). Some lesions showed flaccid pustules and/or blisters at the periphery and yellow crusts in the center of the erythematous plaque (figure 1B). Oral mucosa showed extensive erosions, which appeared approximately two weeks before the admission to our hospital. Her past history was unremarkable, [...]
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IgA Pemphigus with paraneoplastic Pemphigus-like clinical features showing IgA antibodies to desmoglein 1/3 and desmocollin 3, and IgG and IgA antibodies to the basement membrane zone.
Clinical and Experimental Dermatology, 2013Co-Authors: Akihiro Ueda, Ko Temporin, R. Yamazaki, F. Murakami, Mikiko Kyoya, Norito Ishii, Takahiro Hamada, Teruki Dainichi, Shunpei Fukuda, Chihiro SaitoAbstract:Summary A 79-year-old Japanese woman presented with severe recalcitrant erosions on her oral mucosa, resembling paraneoplastic Pemphigus. Using indirect immunofluorescence, we detected IgA antibodies against the cell surface, and both IgG and IgA antibodies against the basement membrane zone. Immunoblotting showed that the IgG antibodies reacted weakly with bullous pemphigoid 230 and periplakin, whereas the IgA antibodies did not react with any antigen. IgA antibodies to both desmoglein (Dsg)1 and Dsg3 were detected by ELISA. IgA antibodies to desmocollin (Dsc)3 were also detected by using cDNAs for human Dsc1–3 transfected into COS-7 cells. Despite treatment with oral prednisolone, high-dose intravenous immunoglobulin and double-filtration plasmapheresis, the skin lesions remained active, and the patient died from bronchiolitis obliterans-like respiratory failure. Despite extensive investIgAtions and postmortem examination, no underlying neoplasms were found. The complex immunopathological findings probably played an important role in the development of the patient's unusual clinical features.
