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Dermatol Ther 2021 Jun 18:e15032. Epub 2021 Jun 18.

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 Apr 20. 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

Ital J Dermatol Venerol 2021 Apr 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

Front Immunol 2020 22;11:1614. Epub 2020 Jul 22.

Deparment of Dermatology, Rouen University Hospital, Center de référence des maladies bulleuses auto-immunes, Normandie University, Rouen, France.

J Allergy Clin Immunol 2020 07;146(1):8-16

Division of Dermatology and Venereology, Department of Medicine Solna, and Center for Molecular Medicine, Karolinska Institutet, Stockholm, Sweden; Unit of Dermatology, Karolinska University Hospital, Department of Dermatology and Venereology, Stockholm, Sweden; Department of Dermatology and Allergy, Technical University of Munich, Munich, Germany. Electronic address:

Autoimmune diseases are driven by either T cells or antibodies reacting specifically to 1 or more self-antigens. Although a number of self-antigens associated with skin diseases have been identified, the causative antigen(s) remains unknown in the great majority of skin diseases suspected to be autoimmune driven. Model diseases such as pemphigus, dermatitis herpetiformis, and more recently psoriasis have added greatly to our understanding of skin autoimmunity. Read More

Pathologe 2020 Jul;41(4):317-325

Klinik für Dermatologie, Allergologie und Venerologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Blisters and erosions of skin and mucous membranes are key features of the clinically heterogeneous group of autoimmune bullous diseases (AIBDs). These can be divided into pemphigoid diseases with autoantibodies against structural proteins of the dermal-epidermal junction, pemphigus diseases with autoantibodies against desmosomal proteins, and dermatitis herpetiformis with autoantibodies against transglutaminases 1 and 2. A differentiation based only on clinical features is often not sufficient. Read More

J Eur Acad Dermatol Venereol 2020 Dec 7;34(12):2890-2897. Epub 2020 Jul 7.

Department of Dermatology, Faculdade de Medicina FMUSP, Universidade de São Paulo, São Paulo, SP, Brazil.

Background: Pemphigus herpetiformis (PH) is a rare clinical subtype of pemphigus with the presence of urticarial plaques, severe pruritus, rare acantholysis and eosinophilic spongiosis.

Objectives: The aim of this study was to investigate the influence of IL-31 and pro-inflammatory cytokines/chemokines in the pathogenesis of PH.

Methods: Twenty-five patients with PH and three groups: pemphigus foliaceus (PF = 14), pemphigus vulgaris (PV = 15) and healthy controls (HC = 20) were selected for this study. Read More

Am J Clin Dermatol 2020 Aug;21(4):525-539

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Eosinophilic dermatoses encompass a broad spectrum of diseases of different etiologies hallmarked by eosinophilic infiltration of the skin and/or mucous membranes, with or without associated blood eosinophilia. The wide range of dermatological manifestations of this spectrum, including nodules and plaques, pustules, blisters, ulcers, and urticarial lesions, is reflected in a non-univocal classification system. We identified six groups of eosinophilic dermatoses based on the predominant anatomic level of involvement: (1) epidermal; (2) of the dermal-epidermal junction; (3) dermal; (4) of the hypodermis and muscle fascia; (5) of the pilosebaceous unit; and (6) vascular/perivascular. Read More

Am J Dermatopathol 2020 Feb;42(2):144-145

Pathology, School of Medicine, University of Virginia, Charlottesville, VA.

Indian Dermatol Online J 2019 Nov-Dec;10(6):727-729. Epub 2019 Nov 1.

Department of Pathology, Lady Hardinge Medical College and Associated Hospital, New Delhi, India.

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

Br J Dermatol 2020 03 8;182(3):802-804. Epub 2019 Nov 8.

Department of Dermatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, 100730, China.

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

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

Int J Dermatol 2019 11 8;58(11):e216-e217. Epub 2019 Jul 8.

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

Front Immunol 2019 19;10:1387. Epub 2019 Jun 19.

Laboratory of Cutaneous Biology, Department of Surgical, Medical, Dental and Morphological Sciences, University of Modena and Reggio Emilia, Modena, Italy.

Pemphigus vulgaris (PV) is a life-threatening mucocutaneous autoimmune blistering disease. It is often associated with autoantibodies to the desmosomal adhesion proteins Desmoglein 3 (DSG3) and Desmoglein 1 (DSG1). Recently, auto-antigens, such as desmocollins and others have been described in PV and in atypical pemphigus forms such as Pemphigus Herpetiformis (PH), Pemphigus Vegetans (PVeg), and Paraneoplastic Pemphigus (PP). Read More

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

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

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

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

Dermatol Clin 2019 Apr 14;37(2):215-228. Epub 2019 Feb 14.

Department of Medicine, University of Central Florida, Health Sciences Campus at Lake Nona, 2627 Northampton Avenue, Orlando, FL 32827-7408, USA. Electronic address:

The treatment of refractory autoimmune blistering diseases (AIBDs) has always been a challenge. Because randomized controlled trials are lacking, treatment has been based on analysis of anecdotal data. The last 2 decades has seen the use of rituximab become a conventional treatment in the therapeutic armamentarium of AIBDs, leading to its Food and Drug Administration indication for pemphigus vulgaris in 2018. Read More

Front Med (Lausanne) 2018 2;5:296. Epub 2018 Nov 2.

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

Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes. Some clinical signs are typical for a specific AIBD, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. The gold standard for diagnosis of AIBD is the detection of autoantibodies or complement component 3 by direct immunofluorescence (DIF) microscopy of a perilesional biopsy. Read More

J Dtsch Dermatol Ges 2018 Nov;16(11):1339-1358

Department of Dermatology and Allergology, Marburg University Medical Center, Marburg, Germany.

Pathophysiologically, bullous autoimmune dermatoses are caused by autoantibodies directed against adhesion molecules or structural proteins of the skin and mucous membranes, clinically resulting in blister formation. Depending on the respective target proteins of the autoimmune response and their location in the skin, a distinction is made between intraepidermal (pemphigus disorders), junctional (pemphigoid disorders), and subepidermal (epidermolysis bullosa acquisita, dermatitis herpetiformis) autoimmune blistering diseases. The most common bullous autoimmune dermatosis, bullous pemphigoid is characterized by marked clinical variability and intense pruritus. Read More

J Dtsch Dermatol Ges 2018 Sep;16(9):1077-1092

Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck.

Blasenbildende Autoimmundermatosen (BAIDs) sind eine heterogene Gruppe seltener Erkrankungen, die klinisch durch Erosionen und/oder Blasen an Haut und Schleimhäuten charakterisiert sind. BAIDs können in zwei Gruppen eingeteilt werden: Pemphigus-Erkrankungen, die durch intraepidermale Blasenbildung und Autoantikörper gegen desmosomale Proteine wie Desmoglein (Dsg) 1, Dsg3 und Mitglieder der Plakin-Familie charakterisiert sind, sowie subepidermale BAIDs, die Pemphigoid-Erkrankungen und die Dermatitis herpetiformis umfassen. Bei der Dermatitis herpetiformis greifen die Autoantikörper die Transglutaminasen 2 und 3 an, während sie bei Pemphigoid-Erkrankungen gegen Strukturproteine der dermoepidermalen Junktionszone gerichtet sind. Read More

J Dtsch Dermatol Ges 2018 Sep;16(9):1077-1091

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

Autoimmune bullous disorders (AIBDs) are a heterogeneous group of rare diseases clinically characterized by erosions and/or blisters on the skin and mucous membranes. AIBDs can be categorized into two groups: pemphigus diseases, characterized by intraepidermal blistering and autoantibodies against desmosomal proteins such as desmoglein (Dsg) 1, Dsg3, members of the plakin family, and subepidermal AIBDs, comprised of pemphigoid diseases and dermatitis herpetiformis. Autoantibodies in dermatitis herpetiformis target transglutaminases 2 and 3, while in pemphigoid disease, autoantibodies are directed against structural proteins of the dermal-epidermal junction. Read More