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Hum Pathol 2022 Jun 25. Epub 2022 Jun 25.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA; Department of Dermatology, Mayo Clinic, Rochester, MN, USA. Electronic address:

Autoimmune bullous dermatoses are characterized by the presence of tissue-bound and often circulating pathogenic autoantibodies targeting structural components of the skin and/or mucous membranes. The diagnostic workup for this heterogeneous group of disorders consists of a multi-step process, of which the light microscopic examination is a crucial component. This review is organized following a classification scheme that is based on two main histopathologic features, namely level of intraepithelial split and composition of the inflammatory infiltrate. Read More

J Clin Med 2022 Jun 19;11(12). Epub 2022 Jun 19.

Department of Dermatology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 807378, Taiwan.

Autoimmune bullous skin disorders are a group of disorders characterized by the formation of numerous blisters and erosions on the skin and/or the mucosal membrane, arising from autoantibodies against the intercellular adhesion molecules and the structural proteins. They can be classified into intraepithelial or subepithelial autoimmune bullous dermatoses based on the location of the targeted antigens. These dermatoses are extremely debilitating and fatal in certain cases, depending on the degree of cutaneous and mucosal involvement. Read More

Int Immunopharmacol 2022 Jun 16;110:108923. Epub 2022 Jun 16.

Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of Medicine, Iran University of Medical Sciences, Tehran, Iran; Skin and Stem Cell Research Center, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:

Janus kinases (JAKs) are a group of intracytoplasmic tyrosine kinase proteins that bind to the cytoplasmic part of the transmembrane cytokine receptors and regulate signaling. The pathophysiology of various autoimmune and autoinflammatory conditions relies on JAK/STAT signaling and therefore, the inhibition of JAK/STAT pathways can be a promising treatment for such diseases, especially inflammatory skin conditions. The current study aimed to evaluate the efficacy of JAK inhibitors in the treatment of immunobullous diseases, including pemphigus, pemphigoid, dermatitis herpetiformis, and epidermolysis bullosa. Read More

Dermatol Pract Concept 2022 May 1;12(2):e2022116. Epub 2022 Apr 1.

EUROIMMUN US Inc, 1 Bloomfield Ave, Mountain Lakes, New Jersey, United States.

Autoimmune bullous diseases (AIBDs) are a group of skin-related disorders that involve damage to structures maintaining cell-cell adhesion, such as desmosomes and hemidesmosomes. Key AIBDs include pemphigus related diseases, pemphigoid related conditions, acquired epidermolysis bullosa (EBA), and dermatitis herpetiformis (DH). Each group of conditions exhibits characteristic clinical lesion patterns and is associated with specific autoantibodies targeting epidermal and dermal structures involved in cell-cell adhesion and skin integrity. Read More

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

J Eur Acad Dermatol Venereol 2022 Apr 1. Epub 2022 Apr 1.

Department of dermatology, CHD Vendée, La Roche Sur Yon, France.

Clin Case Rep 2022 Mar 17;10(3):e05567. Epub 2022 Mar 17.

Dermatology Department Hedi Chaker Hospital Sfax Tunisia.

Pemphigus herpetiformis (PH) is a rare form of pemphigus, especially when occurring in childhood. Misdiagnosis is common in this age group. The disease exhibits diverse clinical and histological aspects. Read More

J Toxicol Pathol 2022 Jan 16;35(1):103-106. Epub 2021 Oct 16.

Translational Research Division, Chugai Pharmaceutical Co., Ltd., 1-135 Komakado, Gotemba, Shizuoka 412-8513, Japan.

Pemphigus is an autoimmune blistering disease characterized by lesions on the skin and mucous membranes. To date, no spontaneous cases of this disease have been reported in cynomolgus monkeys. This report describes the histopathological characteristics of spontaneous pemphigus in a cynomolgus monkey. Read More

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

BMJ Open 2021 12 31;11(12):e053133. Epub 2021 Dec 31.

Skin and Allergy Hospital, Helsinki, Uusimaa, Finland.

Objectives: Many comorbidities are associated with adult asthma and may exacerbate the asthma burden of disease. This study aims to investigate the risk for major oral diseases or oral-manifesting diseases in asthmatic compared with non-asthmatic adults.

Design: We conducted a population-based matched cohort study with a 13. Read More

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

Clin Dermatol 2022 Mar-Apr;40(2):156-165. 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

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

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

Clin Cosmet Investig Dermatol 2021 27;14:1125-1129. Epub 2021 Aug 27.

Department of Dermatology, The Fifth People's Hospital of Hainan Province, Branch of National Clinical Research Center for Skin and Immune Disease, Haikou, 570206, People's Republic of China.

Reports of immune-related adverse events caused by programmed cell death protein-1 inhibitor are becoming increasingly frequent. Herein, we report the first case of pemphigus herpetiformis-type drug reaction presented after the treatment of tislelizumab (6 cycles) in a primary non-small cell lung carcinoma patient. A 56-year-old Chinese man was referred to our department for pruritic annulare erythema and blister for two weeks. Read More

Eur J Dermatol 2021 Jun;31(3):433-435

Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Skinmed 2021;19(3):226-227. Epub 2021 Jun 1.

Department of Dermatology & STD, Vardhman Mahavir Medical College & Safdarjung Hospital, New Delhi, India.

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

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

Acta Dermatovenerol Alp Pannonica Adriat 2021 Jun;30(2):87-88

School of Medicine, National University of Trujillo (FMUNT), Trujillo, Peru.

Pemphigus herpetiformis (PH) is a rare and unique clinical form of pemphigus foliaceus and pemphigus vulgaris. Patients show autoantibodies against desmoglein 1 and less frequently against desmoglein 3 and desmocollins. We report a 24-year-old woman with a 3-year history of recurrent intensely pruritic erythematous papules and annular plaques localized on the trunk and extremities. Read More

Dermatol Ther 2021 09 14;34(5):e15032. Epub 2021 Jul 14.

Department of Specialized, Clinical and Experimental medicine, Division of Dermatology, University of Bologna, Italy.

Autoimmune bullous diseases are a heterogeneous group of diseases characterized by the development of cutaneous and mucosal vesicles, blisters, and finally erosions. The common pathogenetic mechanism is the presence of autoantibodies targeting structural proteins of the skin and mucous membranes (demosomes and hemidesmosomes): in the case of pemphigus, the antigens are intraepidermal, whereas in the case of pemphigoid, dermatitis herpetiformis, and epidermolysis bullosa acquisita they are subepidermal. Mucosal involvement typically affects the oral and ocular mucosa, but in some cases, the upper airways or the upper digestive tract are affected. Read More

Clin Med (Lond) 2021 05;21(3):162-165

St John's Institute of Dermatology, London, UK

Immunobullous diseases are blistering cutaneous disorders that are caused by pathogenic antibodies binding to protein targets within the skin. There are a range of immunobullous disorders with characteristic morphology that relates to the structural properties of the target protein. In this article we will describe the pathogenesis, clinical features and treatment of the most common immunobullous disorders. Read More

J Am Acad Dermatol 2021 08 20;85(2):287-298. Epub 2021 Apr 20.

Division of Dermatology, Department of Internal Medicine, The Ohio State University, Columbus, Ohio. Electronic address:

Despite the expansion of available in vitro laboratory tests at a rate far exceeding that of dermatologic pharmaceuticals, the existing literature is dominated by discussion of the latter. With the advent of numerous new tests, it can be difficult for practicing dermatologists to stay up-to-date on the available options, methodologies, and recommendations for when to order one test over another. Understanding the inherent strengths and weaknesses of these options is necessary to inform appropriate ordering and proper interpretation of the results. Read More

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

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

J Am Acad Dermatol 2021 Jun 5;84(6):1523-1537. Epub 2021 Mar 5.

Epiphany Dermatology, Dallas, Texas. Electronic address:

Intraepithelial autoimmune blistering dermatoses are a rare group of skin disorders characterized by disruptions of inter-keratinocyte connections within the epidermis through the action of autoantibodies. The second article in this continuing medical education series presents validated disease activity scoring systems, serologic parameters of disease, treatments, and clinical trials for pemphigus and its subtypes. Read More

Afr Health Sci 2020 Jun;20(2):885-890

Pandit Deendayal Upadhyay Medical College.

Vesicobullous lesions of skin may occur in different forms of dermatosis, which include various inflammatory, infective, autoimmune, drug induced as well as genetic conditions. Autoimmune bullous lesions, may be fatal if not treated with appropriate agents. Bearing in mind, the morbidity of these diseases, it is important to establish a firm diagnosis. Read More

Am J Dermatopathol 2021 Oct;43(10):689-699

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

Abstract: Immunohistochemistry (IHC) on formalin-fixed, paraffin-embedded tissue has been proposed as a potential tool in the diagnosis of autoimmune bullous diseases (AIBDs) in lieu of standard direct immunofluorescence (DIF) microscopy. To comprehensively determine the diagnostic accuracy of immunoglobulin and complement IHC for diagnosis of AIBDs, we conducted a systematic review and multivariate Bayesian model-based meta-analysis of the literature. Quality and heterogeneity assessment of studies was performed using the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) checklist and the I2 index, respectively. Read More

Clin Exp Dermatol 2021 Jan 10;46(1):207-209. Epub 2020 Oct 10.

Department of Pathology, Western General Hospital and The University of Edinburgh, Edinburgh, UK.

Ital J Dermatol Venerol 2021 04 9;156(2):134-141. Epub 2020 Oct 9.

Department of Dermatology, University of California, Irvine, CA, USA -

In this review, we recap current knowledge about non-desmoglein autoantigens in atypical forms of autoimmune pemphigus. More than 50 keratinocyte proteins, including adhesion molecules, receptors and enzymes as well as mitochondrial proteins can be targeted, leading to alterations in numerous intracellular signaling pathways. Patients with pemphigus herpetiformis feature various combinations of antibodies to desmogleins 1 and 3 and desmocollins 1-3. Read More