459 results match your criteria Pemphigus IgA


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 Apr 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 2020 Aug 13. 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

Prevalence and age distribution of pemphigus and pemphigoid diseases among paediatric patients in Germany.

J Eur Acad Dermatol Venereol 2020 Nov 29;34(11):2600-2605. Epub 2020 Jun 29.

Department of Dermatology, University of Lübeck, Lübeck, Germany.

Background: Autoimmune bullous diseases are rare and mostly occur in adults. Several cases and small case series have been described in children, but no systematic study about the prevalence of autoimmune bullous diseases (AIBD) in children is available.

Patients And Methods: We analysed data of 1. Read More

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November 2020

A case of tinea incognito mimicking subcorneal pustular dermatosis.

Dermatol Online J 2020 Feb 15;26(2). Epub 2020 Feb 15.

Department of Dermatology, School of Medicine, Ankara Yıldırım Beyazıt University, Ankara.

An 89-year-old woman presented with plaque-like lesions, accompanied with pustules and desquamation on the back and front of the trunk for approximately one year. Long term use of potent topical corticosteroids was ineffective. Because of the chronicity of her condition, the diagnoses of subcorneal dermatosis or subcorneal pustular dermatosis type of IgA pemphigus were considered. Read More

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February 2020

Cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcoma.

Gynecol Oncol Rep 2020 May 18;32:100534. Epub 2020 Jan 18.

University of Virginia Health System, 1215 Lee St, Charlottesville, VA 22903, United States.

Pemphigus is a group of autoimmune intraepidermal blistering diseases caused by immunoglobulins directed against keratinocyte cell surface components. In this case report, we identify a non-classical paraneoplastic pemphigus (PNP) foliaceous related to an undifferentiated uterine sarcoma. The patient is a 54-year-old Chinese female with a past medical history of arthritis who presented with worsening fatigue in November 2017 and an itchy, blistering, erythematous annular plaque that first appeared on her chest in February 2018. Read More

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IgA pemphigus: A systematic review.

J Am Acad Dermatol 2020 Jun 5;82(6):1386-1392. Epub 2019 Dec 5.

Department of Dermatology, University of Illinois at Chicago, Chicago, Illinois. Electronic address:

Background: The clinical, histologic, and immunopathologic features of IgA pemphigus have not been studied on a large scale.

Objective: To synthesize existing data on the epidemiologic, clinical, histologic, and immunologic features of IgA pemphigus.

Methods: We performed a systematic review using MEDLINE, Embase, and Web of Science databases. Read More

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Aetiopathogenesis of severe cutaneous adverse reactions (SCARs) in children: A 9-year experience in a tertiary care paediatric hospital setting.

Clin Exp Allergy 2020 01 28;50(1):61-73. Epub 2019 Oct 28.

Allergy Unit, Department of Paediatrics, Anna Meyer Children's University Hospital, Florence, Italy.

Background: Severe cutaneous adverse reactions (SCARs) are delayed-type hypersensitivity reactions to drugs including as follows: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), Stevens-Johnson syndrome (SJS), Toxic Epidermal Necrolysis (TEN) and Acute Generalized Exanthematous Pustulosis (AGEP). Incidence, triggers and management of SCARs have not been investigated in large-scale epidemiological studies on children.

Objective: The aim of our study was to collect epidemiological, clinical and aetiological data from children with SCARs referred to our tertiary care paediatric hospital of Florence. Read More

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January 2020

Alopecia in Autoimmune Blistering Diseases: A Systematic Review of Pathogenesis and Clinical Features of Disease.

Skin Appendage Disord 2019 Aug 10;5(5):263-275. Epub 2019 Jul 10.

Department of Dermatology, St. George Hospital, Sydney, New South Wales, Australia.

Background: Autoimmune blistering diseases (AIBD) are characterised by the body's production of autoantibodies against structural proteins in the epidermis and/or the basement membrane on cutaneous and mucosal surfaces. Alopecia is a complication of AIBD that has generally been overlooked in patients with severe blistering diseases because it is regarded as a cosmetic issue. Yet recent research into quality of life tools has found that stigmatisation by appearance plays a significant role in blistering diseases. Read More

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Systematic Review of Safety and Efficacy of Rituximab in Treating Immune-Mediated Disorders.

Front Immunol 2019 6;10:1990. Epub 2019 Sep 6.

Department of Immunology, University Hospital Zurich, Zurich, Switzerland.

During the past years biologic agents (also termed biologicals or biologics) have become a crucial treatment option in immunological diseases. Numerous articles have been published on biologicals, which complicates the decision making process on the use of the most appropriate biologic for a given immune-mediated disease. This systematic review is the first of a series of articles assessing the safety and efficacy of B cell-targeting biologics for the treatment of immune-mediated diseases. Read More

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October 2020

Bullous Diseases in Children: A Review of Clinical Features and Treatment Options.

Paediatr Drugs 2019 Oct;21(5):345-356

Department of Dermatology, University of Minnesota, 240 Phillips-Wangensteen Building, 516 Delaware Street Southeast, Minneapolis, MN, 55455, USA.

Bullous diseases are uncommon in children; however, as they have the potential to affect quality of life, occasionally have long-term side effects in the setting of scarring processes, and carry a rare risk of underlying malignancy [e.g., with paraneoplastic pemphigus (PNP)], knowledge of their clinical presentation and treatment options is essential. Read More

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October 2019

Complement Activation in Autoimmune Bullous Dermatoses: A Comprehensive Review.

Front Immunol 2019 26;10:1477. Epub 2019 Jun 26.

Department of Pathology, Erasmus Medical Center Rotterdam, Rotterdam, Netherlands.

Autoimmune bullous dermatoses (AIBD) are characterized by circulating autoantibodies that are either directed against epidermal antigens or deposited as immune complexes in the basement membrane zone (BMZ). The complement system (CS) can be activated by autoantibodies, thereby triggering activation of specific complement pathways. Local complement activation induces a pathogenic inflammatory response that eventually results in the formation of a sub- or intraepidermal blister. Read More

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October 2020

Ocular Manifestations and Management of Autoimmune Bullous Diseases.

J Ophthalmic Vis Res 2019 Apr-Jun;14(2):195-210

Ocular Tissue Engineering Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Autoimmune bullous diseases with ocular involvement consist of a group of systemic entities that are characterized by formation of autoantibodies against the proteins of the epithelial basement membrane zone of the conjunctiva. Mostly, the elderly are affected by these diseases. The characteristic patterns of mucocutaneous involvement and the specific tissue components targeted by these autoantibodies are differentiating features of these diseases. Read More

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Mucous membrane pemphigoid and oral blistering diseases.

Clin Exp Dermatol 2019 Oct 18;44(7):732-739. Epub 2019 May 18.

Oral Medicine, Dental Institute, Guy's and St Thomas' NHS Foundation Trust, London, UK.

The autoimmune blistering disorders present with variable frequency in the oral cavity. Recognition of their key clinical features at presentation is important, as there are many causes of oral ulceration. Careful history-taking, clinical examination, an understanding of pathogenesis and appropriate investigations are essential. Read More

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October 2019

Oral autoimmune vesicobullous diseases: Classification, clinical presentations, molecular mechanisms, diagnostic algorithms, and management.

Periodontol 2000 2019 06;80(1):77-88

Oral Medicine Unit, Department of Neurosciences, Reproductive and Odontostomatological Sciences, Federico II University of Naples, Naples, Italy.

Mucocutaneous blistering autoimmune diseases are a group of systemic, rare, chronic disorders characterized by humoral-mediated immunologic mechanisms against epithelial, basement membrane, and subepithelial tissues. Morbidity and mortality can be completely different among these diseases, with outcome being dependent on an early and accurate diagnosis, systemic comorbidities, and the patient's response to treatment. Definitive diagnosis is based on clinical and histopathologic findings. Read More

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Review of autoimmune blistering diseases: the Pemphigoid diseases.

J Eur Acad Dermatol Venereol 2019 Sep 11;33(9):1685-1694. Epub 2019 Jul 11.

St George Hospital, Sydney, Australia.

Autoimmune Blistering Diseases of the Pemphigoid type is characterised by sub-epidermal blisters (SEB) with circulating autoantibodies against components of the basement membrane zone (BMZ). The main disorders to date include bullous pemphigoid (BP), pemphigoid gestationis, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), linear IgA disease (LABD), dermatitis herpetiformis (DH), lichen planus pemphigoides and bullous lupus. This is in contrast to pemphigus and related disorders, which demonstrate intraepidermal acantholysis and a positive Nikolsky sign. Read More

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September 2019

Detection of IgG Autoantibodies against Desmocollin-3 in Greek Patients with Pemphigus.

Acta Dermatovenerol Croat 2019 Mar;27(1):8-10

Parthena Meltzanidou, MSc, PhD candidate, Molecular Biology Laboratory, Aristotle University School of Medicine, Papageorgiou General Hospital, 57500 Thessaloniki, Greece;

Pemphigus is an autoimmune bullous disorder caused by autoantibodies against desmosomal cadherins. The most common clinical forms are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Among the numerous proteins that are considered responsible for the cohesion of keratinocytes in epidermis, desmocollin-3 (Dsc-3) has been initially reported to participate in epidermal blistering in mice. Read More

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MPGN Type 3 Associated with Pemphigus Herpetiformis Mimicking PGNMID and Dermatitis Herpetiformis.

Case Rep Nephrol Dial 2019 Jan-Apr;9(1):15-24. Epub 2019 Mar 21.

Department of Nephrology, Juntendo University Faculty of Medicine, Tokyo, Japan.

A 45-year-old man suffering from dermal blistering disease with proteinuria and hematuria underwent renal biopsy. The renal biopsy specimen suggested proliferative glomerulonephritis with monoclonal IgG deposits under routine light, immunofluorescence and electron microscopy. The staining for IgG subclasses (IgG1 and IgG2) and κ/λ light chain indicated secondary immune complex type MPGN type 3. Read More

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[Autoimmune blistering dermatoses in children].

Hautarzt 2019 Apr;70(4):277-282

Klinik für Dermatologie und Venerologie, Universitätsklinikum Freiburg, Medizinische Fakultät, Albert-Ludwigs-Universität Freiburg, Hauptstr. 7, 79104, Freiburg, Deutschland.

Autoimmune blistering skin disorders represent a rare group of autoantibody-induced dermatoses against desmosomal and hemidesmosomal molecules. The common age of onset for pemphigus and pemphigoid, as well as dermatitis herpetiformis, encompasses the adult age, but all these disorders can be observed neonatally and/or during childhood. If the disease occurs postpartum or neonatally, physicians should consider transplacental transmission of pathogenic maternal immunoglobulin G (IgG)-autoantibodies, and both mother and child should be included in the diagnostic work up. Read More

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[IGA Pemphigus].

Presse Med 2019 Apr 27;48(4):461-464. Epub 2019 Mar 27.

Hôpital militaire d'instruction Mohammed V, service de dermatologie, 10000 Hay Riad, Rabat, Maroc.

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Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria.

Int J Dermatol 2019 Sep 22;58(9):997-1007. Epub 2019 Mar 22.

Department of Dermatology, Mayo Clinic, Jacksonville, FL, USA.

Pemphigus herpetiformis (PH), a rare type of pemphigus, is characterized by immunologic findings consistent with pemphigus but with a unique clinical and pathologic presentation. PH was first described as resembling dermatitis herpetiformis clinically, but because of its variable presentation, it can also resemble linear immunoglobulin A bullous dermatosis and bullous pemphigoid. We reviewed reported cases to analyze the most frequent clinical, pathologic, and immunologic characteristics and to propose corresponding diagnostic criteria. Read More

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September 2019

First presentation of Sneddon-Wilkinson disease with unexpected immunoglobulin A gammopathy: A case report and review of the literature.

SAGE Open Med Case Rep 2019 30;7:2050313X19826432. Epub 2019 Jan 30.

University of Saskatchewan, Saskatoon, SK, Canada.

We present a case of Sneddon-Wilkinson disease in a 52-year-old female at her first presentation to dermatology. Outlined in the case are various investigations undertaken at this initial presentation, including rheumatologic and hematologic malignancy markers, which identified immunoglobulin A gammopathy. The systemic and topical therapies used to treat the patient's condition are described, as well as her response to these treatments. Read More

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January 2019