385 results match your criteria Pemphigus IgA


Annular bullous pemphigoid: A case report and review of literature.

SAGE Open Med Case Rep 2022 21;10:2050313X221093128. Epub 2022 Apr 21.

Division of Dermatology, Hôpital du Sacré-Cœur de Montréal, Université de Montréal, Montreal, QC, Canada.

Bullous pemphigoid is an autoimmune blistering disease that primarily affects the geriatric population. It often presents as urticarial erythematous plaques, which evolve into subepidermal blisters accompanied by pruritus. Although rare, clinical variants of bullous pemphigoid have been documented. Read More

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Autoimmune mucocutaneous blistering diseases after SARS-Cov-2 vaccination: A Case report of Pemphigus Vulgaris and a literature review.

Pathol Res Pract 2022 Apr 5;232:153834. Epub 2022 Mar 5.

Department of Neurosciences, Reproductive and Odontostomatological Sciences, Federico II University of Naples, via Pansini 5, 80131 Naples, Italy.

Background: Cases of severe autoimmune blistering diseases (AIBDs) have recently been reported in association with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination.

Aims: To describe a report of oropharyngeal Pemphigus Vulgaris (OPV) triggered by the mRNABNT162b2 vaccine (Comirnaty®/ Pfizer/ BioNTech) and to analyze the clinical and immunological characteristics of the AIBDs cases reported following the SARS-CoV-2 vaccination.

Methods: The clinical and immunological features of our case of OPV were documented. Read More

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Utility of IgG4 immunohistochemistry detection in pemphigus diagnosis.

SAGE Open Med Case Rep 2022 19;10:2050313X211072982. Epub 2022 Jan 19.

Consulta de Dermatología, Luminox Skin Centre, Torre Noox, Ciudad de México, México.

Pemphigus includes a group of blistering autoimmune diseases that affect the skin and mucosa, characterized by the formation of epidermal bullous and the presence of antibodies against binding proteins. Pemphigus is classified according to clinical presentation, target molecule, and IgG production as pemphigus vulgaris, foliaceous, IgA-pemphigus, and paraneoplastic pemphigus. Thus, the identification of autoantibodies class and site of deposition is mandatory. Read More

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January 2022

Diagnostics for Dermatologic Diseases with Autoantibodies.

J Appl Lab Med 2022 01;7(1):165-196

Immunodermatology Laboratory, Department of Dermatology, University of Utah, UT, USA.

Background: Dermatologic diseases with autoantibodies were recognized early as autoimmunity became accepted as a pathogenic immunologic concept. Laboratory testing to identify disease-defining autoantibodies and investigate their role in pathophysiology has evolved since.

Content: Blistering dermatologic diseases, profiled by autoantibody production, target epithelial components critical in cell-cell and cell-matrix adhesion, resulting in epithelial separation and other characteristic features of the disorders. Read More

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January 2022

Epidemiology of Pemphigus.

JID Innov 2021 Mar 20;1(1):100004. Epub 2021 Feb 20.

Lűbeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.

Pemphigus is an epidemiologically heterogeneous group of autoimmune bullous diseases comprising pemphigus vulgaris (PV), pemphigus foliaceus, paraneoplastic pemphigus, IgA pemphigus, and pemphigus herpetiformis. Recently, our knowledge about the frequency of pemphigus, which is highly variable between different populations, has considerably expanded, and the first non-HLA genes associated with PV have been identified. In addition, a variety of comorbidities, including other autoimmune diseases, hematological malignancies, and psoriasis, have been described in this variant. Read More

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Nutrition and bullous diseases.

Clin Dermatol 2021 Nov 19. Epub 2021 Nov 19.

Department of Dermatology, University of Connecticut Health Center, Farmington, Connecticut, USA. Electronic address:

Although relatively uncommon, autoimmune bullous diseases carry the risk of increased mortality and can significantly impact quality of life. This group of diseases is broad and encompasses subepidermal conditions such as bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, dermatitis herpetiformis, and linear IgA bullous dermatosis, as well as intraepidermal conditions such as pemphigus and its variants. The pathophysiology of each condition is incompletely understood but broadly involves the formation of autoantibodies targeting skin adhesion proteins, a process that relies on a complex interplay between a dysregulated immune system, genetic predisposition, and environmental factors. Read More

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November 2021

Conceptualization and validation of an innovative direct immunofluorescence technique utilizing fluorescein conjugate against IgG + IgG4 for routinely diagnosing autoimmune bullous dermatoses.

Cent Eur J Immunol 2021 15;46(2):183-190. Epub 2021 Jun 15.

Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.

Introduction: Autoimmune bullous diseases (ABDs) are potentially life-threatening mucocutaneous illnesses that require diagnosis with direct immunofluorescence (DIF). In this study we compared the diagnostic accuracy of traditional DIF (DIFt; separate immunoglobulin (Ig) G, IgG1, IgG4, IgA, IgM and C3 deposits detection) and modified DIF (DIFm; simultaneous IgG + IgG4 deposits detection instead of separate IgG and IgG4 deposits detection) in routine diagnostics of ABDs.

Material And Methods: Eighteen patients with ABDs (7 with pemphigus dermatoses and 11 with subepithelial ABDs) were evaluated with DIFt and DIFm. Read More

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An Updated Review of Pemphigus Diseases.

Medicina (Kaunas) 2021 Oct 9;57(10). Epub 2021 Oct 9.

Department of Dermatology, College of Medicine, University of Florida, Gainesville, FL 32606, USA.

Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus represent the spectrum of autoimmune bullous dermatoses of the pemphigus family. The pemphigus family of diseases is characterized by significant morbidity and mortality. Read More

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October 2021

IgM Deficiency in Autoimmune Blistering Mucocutaneous Diseases Following Various Treatments: Long Term Follow-Up and Relevant Observations.

Front Immunol 2021 27;12:727520. Epub 2021 Sep 27.

Department of Dermatology, The Center for Blistering Diseases, Tufts University School of Medicine, Boston, MA, United States.

IgM deficiency has been reported in patients with many autoimmune diseases treated with Rituximab (RTX). It has not been studied, in detail, in autoimmune mucocutaneous blistering diseases (AIMBD). Our objectives were: (i) Examine the dynamics of IgM levels in patients with and without RTX. Read More

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November 2021

A multicentre prospective analysis of the incidence of pemphigoid diseases in Turkey.

Australas J Dermatol 2021 Nov 12;62(4):e496-e503. Epub 2021 Oct 12.

Faculty of Medicine, Department of Dermatology, Karadeniz Technical University, Trabzon, Turkey.

Background: The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our aim in this study is to determine the incidence, demographics and clinical characteristics associated with diseases of the pemphigoid group. Read More

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November 2021

Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review).

Exp Ther Med 2021 Nov 20;22(5):1335. Epub 2021 Sep 20.

Department of Oral Medicine and Oral Dermatology, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iași, Romania.

Pemphigus represents a group of chronic inflammatory disorders characterized by autoantibodies that target components of desmosomes, leading to the loss of intercellular adhesion between keratinocytes and causing intraepithelial blistering. The pemphigus group consists of four main clinical types with several variants: pemphigus vulgaris (with pemphigus vegetans and pemphigus herpetiformis as variants), pemphigus foliaceus, paraneoplastic pemphigus and IgA pemphigus (with two clinical variants: intraepidermal neutrophilic IgA dermatosis and subcorneal pustular dermatosis). Genetic factors are involved in the pathogenesis, with HLA-DR4 (DRB1*0402) and HLA-DRw6 (DQB1*0503) allele more common in patients with pemphigus vulgaris, HLA class II DRB1*0344 and HLA Cw*1445 correlated with paraneoplastic pemphigus, and HLA-DRB1*04:01, HLA-DRB1*04:06, HLA-DRB1*01:01, HLA-DRB1*14, associated with a higher risk of developing pemphigus foliaceus. Read More

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November 2021

Assessment of Clinical and Laboratory Use of the Cutaneous Direct Immunofluorescence Assay.

JAMA Dermatol 2021 Nov;157(11):1343-1348

Massachusetts General Physician Organization Dermatopathology Associates, Newton, Massachusetts.

Importance: Dermatologists submit direct immunofluorescence (DIF) biopsies on a daily basis, using an assay detecting immunoreactant deposition with a panel that has traditionally comprised immunoglobulin (Ig) G, IgA, IgM, C3, and fibrin, with or without albumin antibodies.

Objectives: To evaluate and compare the frequency of immunoreactants in DIF biopsies submitted over an 8-year period and assess use by dermatologists based on clinical impression.

Design, Setting, And Participants: A quality improvement study was conducted in a community outreach reference laboratory associated with a large academic medical center. Read More

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November 2021

Direct immunofluorescence analysis of oral Tzanck smears for pemphigus vulgaris: A diagnostic test.

J Oral Pathol Med 2021 Nov 11;50(10):1050-1056. Epub 2021 Oct 11.

Department of Oral Medicine, Peking University School and Hospital of Stomatology, National Center of Stomatology, National Clinical Research Center for Oral Diseases, National Engineering Laboratory for Digital and Material Technology of Stomatology, Beijing Key Laboratory of Digital Stomatology, Research Center of Engineering and Technology for Computerized Dentistry Ministry of Health, NMPA Key Laboratory for Dental Materials, Beijing, China.

Background: Pemphigus vulgaris (PV) is a rare and potentially fatal autoimmune blistering disease. Direct immunofluorescence (DIF) and histopathological analysis are crucial methods for PV diagnosis, but oral tissue biopsy is difficult to perform because of the fragile characteristics of the oral mucosa. However, no well-designed diagnostic studies addressing the validity of DIF analysis of oral Tzanck smears for the diagnosis of PV exist. Read More

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November 2021

Anti-laminin 332 antibody detection using biochip immunofluorescence microscopy in a real-life cohort of Italian patients with mucous membrane pemphigoid.

Eur J Dermatol 2021 Aug 26. Epub 2021 Aug 26.

Section of Dermatology, Department of health Sciences (DISSAL), University of Genoa, Genoa, Italy.

Mucous membrane pemphigoid (MMP) with anti-laminin 332 autoantibodies may be associated with malignancies, however, current serological assays have considerable limitations. At present, no commercial test for anti-laminin 332 antibodies is available, restricting the diagnosis to specialized laboratories worldwide. Biochip immunofluorescence microscopy has shown promising results in selected cohorts of laminin 332-MMP patients. Read More

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The epidemiology of autoimmune bullous diseases in Sudan between 2000 and 2016.

PLoS One 2021 13;16(7):e0254634. Epub 2021 Jul 13.

Faculty of Dentistry, University of Khartoum, Khartoum, Khartoum State, Sudan.

Objectives: Autoimmune bullous diseases vary in their clinico-epidemiological features and burden across populations. Data about these diseases was lacking in Sudan. We aimed to describe the epidemiological profile and to estimate the burden of autoimmune bullous diseases in Sudan. Read More

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November 2021

Pathogenic Activation and Therapeutic Blockage of FcαR-Expressing Polymorphonuclear Leukocytes in IgA Pemphigus.

J Invest Dermatol 2021 12 8;141(12):2820-2828. Epub 2021 Jul 8.

Luebeck Institute of Experimental Dermatology (LIED), University of Luebeck, Luebeck, Germany; Department of Dermatology, Allergology and Venerology, University of Luebeck, Luebeck, Germany. Electronic address:

Pathomechanisms in IgA pemphigus are assumed to rely on Fc-dependent cellular activation by antigen-specific IgA autoantibodies; however, models for the disease and more detailed pathophysiologic data are lacking. In this study, we aimed to establish in vitro models of disease for IgA pemphigus, allowing us to study the effects of the interaction of anti-keratinocyte IgA with cell surface FcαRs. Employing multiple in vitro assays, such as a skin cryosection assay and a human skin organ culture model, in this study, we present mechanistic data for the pathogenesis of IgA pemphigus, mediated by anti-desmoglein 3 IgA autoantibodies. Read More

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December 2021

Morphological Spectrum of Vesiculobullous Skin Lesions: An Institutional Perspective.

Cureus 2021 May 30;13(5):e15330. Epub 2021 May 30.

Pathology, Liaquat National Hospital and Medical College, Karachi, PAK.

Introduction A vesiculobullous lesion of the skin encompasses a group of dermatological disorders with protean clinicopathological features. They usually occur as a part of the spectrum of various infectious, inflammatory, drug-induced, genetic, and autoimmune disorders. Therefore, accurate diagnosis of these lesions is essential for appropriate management and to reduce the associated morbidity and mortality. Read More

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Safety, Tolerability, and Activity of ALXN1830 Targeting the Neonatal Fc Receptor in Chronic Pemphigus.

J Invest Dermatol 2021 12 12;141(12):2858-2865.e4. Epub 2021 Jun 12.

Department of Dermatology, Duke University Medical Center, Durham, North Carolina, USA.

Pemphigus is a debilitating IgG-mediated autoimmune disease requiring better tolerated, more targeted, and rapid onset therapies. ALXN1830 is a humanized IgG4 antibody that blocks neonatal Fc receptor interactions with IgG. A multicenter, open-label safety and tolerability phase 1b/2 trial (NCT03075904) was conducted in North America from July 2017 to January 2019 and included patients aged ≥18 years with a confirmed diagnosis of pemphigus (vulgaris or foliaceus) and active disease. Read More

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December 2021

Pemphigus Vulgaris After COVID-19: a Case of Induced Autoimmunity.

SN Compr Clin Med 2021 27;3(8):1768-1772. Epub 2021 May 27.

Department of Pathology, Federal University of Pernambuco, Recife, PE Brazil.

Bullae autoimmune diseases can be induced by environmental factors in a genetically susceptible individual, and viruses may be important triggers for this process. Coronavirus disease 2019 (COVID-19) is a multisystemic disease known for developing many types of skin lesions. However, little is known about post-COVID-19 manifestations. Read More

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A Case of Dapsone-induced Mild Methemoglobinemia with Dyspnea and Cyanosis.

Acta Dermatovenerol Croat 2020 Dec;28(4):249-250

Hisayoshi Imanishi, MD, PhD, Division of Dermatology, Daito Central Hospital, 2-1-11 Ono, Daito, Osaka 574-0042, Japan;

Dear Editor, Dapsone is a dual-function drug with antimicrobial and antiprotozoal effects and anti-inflammatory features (1). In dermatology, it is a first choice for conditions such as leprosy, IgA pemphigus, dermatitis herpetiformis, and linear IgA bullous dermatosis, or an adjunctive treatment for, e.g. Read More

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December 2020

Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis.

J Am Acad Dermatol 2021 Jun 5;84(6):1507-1519. Epub 2021 Mar 5.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.

Intraepithelial autoimmune blistering dermatoses are a rare group of skin disorders characterized by the intraepithelial disruption of intercellular connections through the action of autoantibodies. The first article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major intraepithelial autoimmune blistering dermatoses, including pemphigus foliaceus, pemphigus erythematosus, pemphigus herpetiformis, fogo selvagem, pemphigus vulgaris, pemphigus vegetans, drug-induced pemphigus, IgA pemphigus, IgG/IgA pemphigus, and paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome. Read More

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Helicobacter pylori and skin disorders: a comprehensive review of the available literature.

Eur Rev Med Pharmacol Sci 2020 12;24(23):12267-12287

Department of Biomedical, Dental, Morphological and Functional Imaging Sciences, University of Messina, Messina, Italy.

Helicobacter pylori is a Gram-negative bacterium identified for the first time about 30 years ago and commonly considered as the main pathogenic factor of gastritis and peptic ulcer. Since then, it was found to be associated with several gastrointestinal and extra-gastrointestinal diseases. Helicobacter pylori is also associated with many skin disorders including, but not limited to, chronic urticaria, rosacea, lichen planus, atopic dermatitis, psoriasis, pemphigus vulgaris, vitiligo, primary cutaneous MALT-type lymphoma, sublamina densa-type linear IgA bullous dermatosis, primary cutaneous marginal zone B-cell lymphomas and cutaneous T-cell pseudolymphoma. Read More

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December 2020

Immune serological diagnosis of pemphigus.

Ital J Dermatol Venerol 2021 04 23;156(2):151-160. Epub 2020 Nov 23.

Department of Dermatology, Osaka City University Graduate School of Medicine, Osaka, Japan.

Pemphigus is a rare autoimmune blistering disease which manifests with painful erosions and blisters of the skin and mucosa. This disorder is caused by autoantibodies attacking desmosomal proteins, necessary for cell-cell contact stability and epidermal integrity. Desmoglein (Dsg) 1 and Dsg3 are the two major target antigens in pemphigus. Read More

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Intravenous Immunoglobulins in a Series of 32 Rare and Recalcitrant Immune Dermatoses.

Acta Derm Venereol 2020 Oct 21;100(17):adv00298. Epub 2020 Oct 21.

Division of Allergy and Immunology, Charité - Universitätsmedizin Berlin, DE-10117 Berlin, Germany.

Intravenous immunoglobulins are an effective and well-tolerated treatment option for immune dermatological diseases. However, they are primarily used to treat diseases with a severe course and are mostly used off-label. Therefore, it is important to document case series on the use of intravenous immunoglobulins in rare immune dermatological diseases. Read More

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October 2020

Spectrum of autoimmune bullous diseases among children in Kuwait.

Pediatr Dermatol 2021 Jan 11;38(1):50-57. Epub 2020 Oct 11.

As'ad Al-Hamad Dermatology Center, Salmiya, Kuwait.

Background: Autoimmune bullous diseases (AIBD) are rare among children. The data describing the overall spectrum and prognosis of pediatric AIBD (pAIBD) are scarce, and there are no established treatment guidelines.

Objectives: The present study examined the spectrum, clinical characteristics, and long-term prognosis of pAIBD in a tertiary care pediatric dermatology unit. Read More

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January 2021

Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous Pemphigoid.

Front Med (Lausanne) 2020 2;7:472. Epub 2020 Sep 2.

Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.

Pemphigus and pemphigoid diseases are organ-specific autoimmune diseases of the skin and/or mucous membranes, which are caused by autoantibodies targeting structural proteins of the skin. In other autoimmune diseases, a high prevalence of primary antibody deficiencies was noted. Conversely, a high prevalence of autoimmune diseases is reported in patients with primary antibody deficiencies. Read More

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September 2020

A retrospective study on the clinicopathologic features of IgG/IgA pemphigus.

J Am Acad Dermatol 2021 Jul 13;85(1):237-240. Epub 2020 Aug 13.

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York. Electronic address:

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Flame Figures in Linear Immunoglobulin A Bullous Dermatosis Secondary to Parvovirus B19 Infection.

Am J Dermatopathol 2021 Jan;43(1):60-62

Departments of Dermatology, and.

Abstract: Flame figures represent a characteristic but nondiagnostic histological finding in eosinophilic dermatoses. Some bullous autoimmune diseases with a predominant eosinophilic infiltrate, such as bullous pemphigoid, pemphigoid gestationis, and pemphigus vegetans, may show them. However, it is rare to find them in predominant neutrophilic bullous dermatoses such as linear immunoglobulin A. Read More

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January 2021

IgA pemphigus and linear IgA bullous dermatosis in a patient with ulcerative colitis.

Australas J Dermatol 2020 Nov 17;61(4):e443-e445. Epub 2020 May 17.

Division of Dermatology, Department of Medicine, University of Louisville, Louisville, Kentucky, USA.

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November 2020

Detection of circulating anti-keratinocyte autoantibodies in feline pemphigus foliaceus.

Vet Dermatol 2020 Oct 7;31(5):378-e100. Epub 2020 Jul 7.

Center for Comparative Medicine and Translational Research, North Carolina State University, Raleigh, NC, 27606, USA.

Background: Circulating anti-keratinocyte immunoglobulin (Ig)G targeting desmosomal proteins have been identified in people and dogs with pemphigus foliaceus (PF). By contrast, detection attempts in PF-affected cats have been largely unsuccessful.

Hypothesis/objectives: To detect circulating anti-keratinocyte autoantibodies in PF-affected cats and determine their titres and tissue-staining patterns. Read More

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October 2020