G Ital Dermatol Venereol 2018 May 16. Epub 2018 May 16.

Department of Dermatology, Policlinic Vittorio Emanuele Hospital, Catania, Italy -

Clin Dermatol 2018 Mar - Apr;36(2):231-238. Epub 2017 Oct 3.

Department of Dermatovenereology, University Hospital Center Zagreb and University of Zagreb School of Medicine, Zagreb, Croatia. Electronic address:

Autoimmune blistering diseases (AIBD) are a group of chronic diseases affecting the skin and mucous membranes, with different presentation, clinical course, histologic and immunopathologic findings, and different therapeutic approach. Blisters develop as a result of autoantibodies directed against distinct adhesion structures within desmosomes or within the basement membrane zone. The most common AIBD that develops in the elderly is bullous pemphigoid (previously also named "pemphigoid senilis"), but mature patients can also present with other AIBD as mucous membrane pemphigoid, epidermolysis bullosa acquisita, paraneoplastic pemphigus, pemphigus vulgaris, pemphigus foliaceus, linear IgA dermatosis, and dermatitis herpetiformis. Read More

Immunol Res 2018 Apr;66(2):255-270

Department of Dermatology, Rambam Health Care Campus, POB 9602, 31096, Haifa, Israel.

Pemphigus forms a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. This group has a chronic course leading to high morbidity and mortality. It is characterized by the production of pathogenic autoantibodies directed against different proteins of the desmosome, leading histologically to intraepidermal cleavage, and clinically to vesicles and erosions on the epithelium of the mucous membranes and/or the skin. Read More

G Ital Dermatol Venereol 2018 Jan 24. Epub 2018 Jan 24.

Dermatology Unit, Department of Surgery and Intensive Care, IRCCS G. Gaslini Children's Hospital, Genoa, Italy -

Clin Exp Dermatol 2018 Apr 14;43(3):360-362. Epub 2018 Jan 14.

National Skin Centre, Singapore.

An Bras Dermatol 2017 ;92(5 Suppl 1):145-147

Dermatology Outpatient Clinic at Hospital do Açúcar de Alagoas do Centro Universitário Tiradentes, Maceió, Alagoas (AL), Brazil.

Pemphigus herpetiformis is an autoimmune bullous disease, that combines clinical features of dermatitis herpetiformis and linear IgA bullous dermatosis and immunological characteristics of pemphigus, which makes this disease peculiar and this diagnosis rarely suspected in the first evaluation of the patient. The reported case is of a patient with clinically bullous disease similar to dermatitis herpetiformis, whose multiple biopsies were inconclusive, and only after direct immunofluorescence with a pemphigus pattern (intraepidermal intercellular pattern) the confirmation of the diagnosis was possible. Read More

Clin Exp Dermatol 2018 Apr 12;43(3):324-326. Epub 2017 Dec 12.

Department of Dermatology and Venereology, China-Japan Friendship Hospital, No. 2, Yinghua East Street, Chaoyang District, Beijing, 100029, China.

Pol J Pathol 2017;68(2):109-116

Here we investigated the cutaneous CD32A and CD89 expression in relation to the neutrophil elastase (NE) expression and serum level of anti-desmoglein 1 and 3 (DSG1/DSG3) IgG in pemphigus, anti-BP180/BP230 IgG in bullous pemphigoid (BP), anti-gliadin nonapeptides (npG), tissue (tTG), and epidermal transglutaminases (eTG) IgA in dermatitis herpetiformis (DH). The examined material consisted of skin/mucosal tissues and sera. In total, 87 patients were studied. Read More

Int J Dermatol 2017 Oct 30;56(10):996-1002. Epub 2017 Aug 30.

Department of Dermatology, Medical University of Warsaw, Warsaw, Poland.

Background: Scalp is a common location of autoimmune bullous diseases. Trichoscopy is a noninvasive method for diagnosing hair and scalp diseases. Data on trichoscopy in autoimmune bullous diseases are limited to the studies on pemphigus including a small number of patients. Read More

Am J Dermatopathol 2018 Feb;40(2):111-117

Department of Pathology, University of Massachusetts Medical School, UMass Memorial Medical Center, Worcester, MA.

Direct immunofluorescence (DIF) on frozen tissue (DIF-F) is the method of choice for the identification of immune deposits present in skin and other tissues. DIF can also be performed on formalin-fixed paraffin-embedded tissue (DIF-P) after antigen retrieval with proteases and has proven to be of value in renal pathology. However, its utility in skin biopsies has not been fully examined. Read More

Indian J Dermatol 2017 Jul-Aug;62(4):440

Department of Dermatology, School of Medicine, Marmara University, Istanbul, Turkey.

Background: Autoimmune bullous disorders (AIBDs) are a heterogeneous group of diseases which are rarely seen in children. Studies concerning the immunobullous diseases in pediatric patients are scarce.

Aims And Objectives: In this study, we aimed to investigate the clinical features and treatment outcomes of AIBDs in children. Read More

Skinmed 2017 1;15(3):175-180. Epub 2017 Jun 1.

Department of Dermatology, Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

Autoimmune bullous diseases (ABDs) are uncommon but significant skin disorders with relatively high morbidity and mortality. Some surveys have been carried out to describe the spectrum of ABDs in a region, but this is the first that has focused on ABDs in elderly patients. This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients. Read More

Turk J Med Sci 2017 Jun 12;47(3):832-840. Epub 2017 Jun 12.

Department of Dermatology, Faculty of Medicine, Dokuz Eylül University, İzmir, Turkey.

Background/aim: The distribution and the demographic, etiological, and clinical features of autoimmune bullous diseases (ABDs) vary according to geographic regions of the world. The limited number of reported studies in Turkey are mostly retrospective and generally include only pemphigus. The aim of our study was to evaluate the demographic data, clinical features, associated triggering factors, and comorbidities in patients newly diagnosed with ABDs. Read More

Mediators Inflamm 2017 10;2017:8980534. Epub 2017 May 10.

Department of Immunopathology, Chair of Allergy, Immunology and Dermatology, Medical University of Lodz, Lodz, Poland.

Dermatitis herpetiformis (DH), bullous pemphigoid (BP), and pemphigus vulgaris (PV) are autoimmune bullous skin conditions with eosinophilic and neutrophilic infiltrations. While cytokines are crucial for the affinity and activation of different leukocyte cells in the inflammation and blister formation, there are no studies concerning a role of IL-36. The goal of the study was to analyze whether interleukin 36 is involved in pathogenesis of DH, BP, and PV. Read More

Pediatr Dermatol 2017 May;34(3):342-346

Dermatology Program, Division of Immunology and Allergy, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.

Pemphigus herpetiformis (PH) is a rare autoimmune bullous condition usually seen in adults and likely underrecognized in children. We describe a 2-year-old girl who presented with a chronic, generalized, blistering rash consistent with pediatric PH based on histology and immunohistochemistry and review the literature. Prognosis is generally more favorable in children than adults. Read More

J Dermatol 2017 Feb 5;44(2):189-193. Epub 2016 Nov 5.

Department of Dermatology, Kurume University School of Medicine, Kurume, Japan.

Pemphigus herpetiformis (PH) is a rare variant of pemphigus characterized by erythemas and vesicles, tending to present with annular-shaped lesions. Immunologically, immunoglobulin (Ig)G deposition at the keratinocyte cell surfaces is observed. Linear IgA bullous dermatosis (LABD) is a rare subepidermal blistering disease with linear IgA deposits at the epidermal basement membrane zone (BMZ). Read More

Bratisl Lek Listy 2017 ;118(4):243-249

Aims: To determine the diagnostic accordance between histopathological and direct immunofluorescence diagnosis of patients with autoimmune vesiculobullous skin diseases.

Background: The term pemphigus refers to a group of autoimmune blistering diseases mediated by auto-antibodies directed against desmoglein proteins. The differentiation between the various bullous diseases is important for treatment and prognosis. Read More

J Invest Dermatol 2017 Jun 5;137(6):1199-1203. Epub 2017 Apr 5.

Institute of Anatomy and Cell Biology, Ludwig-Maximilians-Universität, Munich, Germany.

Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal blistering (pemphigoid diseases and dermatitis herpetiformis). The Pathogenesis of Pemphigus and Pemphigoid Meeting, organized by the Departments of Dermatology in Lübeck and Marburg and the Institute of Anatomy and Cell Biology, Munich, was held in September 2016 in Munich. The meeting brought together basic scientists and clinicians from all continents dedicating their work to autoimmune blistering diseases. Read More

Am J Clin Dermatol 2017 Apr;18(2):287-297

Departments of Dermatology, Preventive Medicine and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Suite 1600, 676 N. St. Clair St., Chicago, IL, 60611, USA.

Background: Little is known about the epidemiology of pediatric autoimmune blistering disorders (PAIBD).

Objective: We sought to determine the inpatient burden and comorbidities of PAIBD.

Methods: We analyzed data from the Nationwide Inpatient Sample from 2002 to 2012, which contained a representative 20% sample of all US hospitalizations. Read More

J Invest Dermatol 2017 Jan;137(1):e1-e6

Department of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany. Electronic address:

Autoimmune blistering diseases are examples of autoantibody-mediated, organ-specific autoimmune disorders. Based on a genetic susceptibility, such as a strong HLA-class II association, as yet unknown triggering factors induce the formation of circulating and tissue-bound autoantibodies that are mainly directed against adhesion structures of the skin and mucous membranes. Compared with other autoimmune diseases, especially systemic disorders, the pathogenicity of autoimmune blistering diseases is relatively well described. Read More

JAAD Case Rep 2016 Nov 3;2(6):436-438. Epub 2016 Dec 3.

Department of Dermatology, Mayo Clinic, Scottsdale, Arizona.

Cutan Ocul Toxicol 2017 Sep 23;36(3):302-304. Epub 2016 Nov 23.

b Department of Pathology , Ankara Ataturk Training and Research Hospital , Ankara , Turkey.

Drug-related pemphigus is very rare in children. Erdosteine is a thiol compound having mucoactive, antioxidant, anti-inflammatory, and antitussive effects and is reported to be safe for treatment of acute respiratory tract diseases in children. Herein, we report a 9-year-old boy presented with pemphigus herpetiformis associated with anti-desmoglein 1 antibodies due to erdosteine consumption. Read More

Actas Dermosifiliogr 2016 Nov 20;107(9):785-786. Epub 2016 Jun 20.

Unidad de Gestión Clínica de Dermatología Médico-Quirúrgica y Venereología, Hospital Universitario Puerta del Mar, Cádiz, España; DERMACHAT (Grupo Español de Consenso on-line en Dermatología).

J Dtsch Dermatol Ges 2016 Oct;14(10):995-1005

Institute of Social Medicine, Faculty of Medicine, University of Belgrade, Belgrade, Serbia.

Background And Objectives: While most previous surveys on the clinico-epidemiological features of autoimmune bullous diseases (AIBDs) have predominantly focused on a single disease entity or just one disease group, there have been only few studies examining the incidence of various AIBDs. In the present study, we set out to determine the spectrum of AIBDs, to estimate the incidence of the most common AIBDs, and to examine their temporal trends in Central Serbia over a period of 20 years.

Methods: We retrospectively recruited 1,161 new AIBD cases diagnosed in Central Serbia during the period from January 1991 to December 2010. Read More

J Dtsch Dermatol Ges 2016 Oct;14(10):995-1006

Institut für Sozialmedizin, Medizinische Fakultät, Universität Belgrad, Belgrad, Serbien.

Hintergrund Und Ziele: Die meisten früheren Arbeiten zu den klinisch-epidemiologischen Merkmalen von bullösen Autoimmunerkrankungen (AIBD) konzentrierten sich vor allem auf eine einzige Krankheitsentität oder nur eine Krankheitsgruppe; nur in wenigen Studien wurde die Inzidenz verschiedener AIBD untersucht. Bei der vorliegenden Studie war es unser Ziel, das gesamte Spektrum der AIBD zu betrachten, die Inzidenz der häufigsten AIBD zu ermitteln und die zeitlichen Trends ihres Auftretens in Zentralserbien über einen Zeitraum von 20 Jahren zu untersuchen.

Methoden: Wir rekrutierten retrospektiv 1161 AIBD-Fälle, die in Zentralserbien von Januar 1991 bis Dezember 2010 neu diagnostiziert wurden. Read More

Vojnosanit Pregl 2016 Oct;73(10):967-72

Introduction: Pemphigus herpetiformis is the rare variant of pemphigus with characteristic clinical features, histopathological findings different from the convectional pemphigus, and immunological findings consistent with pemphigus.

Case Report: We presented a 65-year-old woman with initial pruritus followed by pruritic urticarial papules and plaques, some with annular rings of tense vesicles on the periphery, on the trunk and extremities, with no mucous lesions. Histopathological examination demonstrated spongiosis and intraepidermal vesicles in the mid or subcorneal epidermis in some biopsy specimen, with neutrophil and eosinophil infiltrate. Read More

J Dermatol Sci 2016 Oct 6;84(1):17-23. Epub 2016 Jul 6.

Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary. Electronic address:

Background: Recently, high prevalence of cryofibrinogenaemia has been observed in plasma of untreated dermatitis herpetiformis (DH) patients, and the pathological IgA and TG3 deposits in the papillary dermis were found to co-localize with fibrin and fibrinogen.

Objective: To study the fibrinolytic potential in plasma of untreated, dapsone and or/gluten-free diet treated DH patients as well as the in vitro effect of dapsone on the fibrinolytic profile.

Method: Plasma samples of 23 DH patients, 19 healthy subjects and 5 pemphigus vulgaris patients were investigated by a turbidimetric-clot lysis assay. Read More

Clin Dermatol 2016 May-Jun;34(3):353-8. Epub 2016 Mar 2.

Department of Dermatology, Warren Alpert Medical School of Brown University, Providence, Rhode Island.

Physiologic alterations of the oral and vulvovaginal mucosal surfaces result from the profound hormonal and immunologic changes of gestation. High estrogen levels are responsible for the vascular changes noted on mucosal surfaces. Gingival hyperemia and edema, gingivitis and pyogenic granuloma are the most common alterations of the oral mucosa during gestation. Read More

J Dermatol 2017 Jan 14;44(1):104-105. Epub 2016 May 14.

Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.

Turk Patoloji Derg 2016 ;32(2):91-8

Department of Pathology Bharati Vidyapeeth Deemed University Medical College and Hospital, SANGLI, INDIA.

Objective: Sub-epidermal bullous disorders belong to immunobullous diseases which develop as a result of autoantibody action against epidermal basement membrane proteins. Clinically, they are tense bullae and do not rupture easily. They are classified into various forms based on histopathology and direct immunofluorescence patterns. Read More

J Invest Dermatol 2016 08 20;136(8):1729-31. Epub 2016 Apr 20.

Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary. Electronic address:

Indian J Dermatol Venereol Leprol 2016 May-Jun;82(3):359

Department of Dermatology, Saudi Hospital, Hajjah, Republic of Yemen.

Background: The incidence of pemphigus, though not documented, seems to be quite high in Yemen. There is no universal consensus on the treatment of this disease.

Aims: The aim was to evaluate the efficacy and side effects of different therapeutic regimens used in patients of pemphigus in North-Western Yemen. Read More

Ann Dermatol 2016 Feb 28;28(1):102-6. Epub 2016 Jan 28.

Department of Dermatology and Cutaneous Biology Research Institute, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.

Pemphigus represents a group of autoimmune blistering diseases caused by autoantibodies against desmogleins (Dsgs), a class of desmosomal cadherins. Recently, several pemphigus patients only with desmocollin (Dsc) 3-specific antibodies have been reported. Here, we report a case of pemphigus herpetiformis (PH), where only anti-Dsc3-specific antibodies but not anti-Dsg antibodies were detected. Read More

Clin Cosmet Investig Dermatol 2016 11;9:15-20. Epub 2016 Jan 11.

Psoriasis Research Center, Department of Dermatology, Farshchian Hospital, Hamadan University of Medical Sciences, Hamadan, Iran.

Background: Autoimmune bullous diseases (ABDs) represent a group of rare, acquired disorders characterized by overlapping features with involvement of the skin and mucous membranes, resistance to treatment, and potential lethality that comprise pemphigus, bullous pemphigoid (BP), epidermolysis bullosa, dermatitis herpetiformis, and linear immunoglobulin A bullous dermatosis.

Aim: The main aim of this study was to identify the epidemiologic characteristics and clinical courses of these common diseases in Hamadan, Iran. Few surveys have been carried out to demonstrate the whole spectrum of ABDs in the literature. Read More

Int J Dermatol 2016 Jun 29;55(6):599-607. Epub 2015 Dec 29.

Department of Dermatology, Mayo Clinic, Rochester, MN, USA.

Autoimmune bullous diseases, such as pemphigus, pemphigoid, and dermatitis herpetiformis, are uniquely associated with vulnerability in the mucocutaneous barrier against infection. The management of immunobullous diseases is complex and may at times require immunosuppressive medications. Iatrogenic immunosuppression may increase susceptibility to vaccine-preventable illnesses. Read More

Clin Dermatol 2015 Nov-Dec;33(6):644-56. Epub 2015 Sep 14.

Departments of Dermatology & Pediatrics, Columbia University College of Physicians and Surgeons, 161 Fort Washington Avenue, 12th Floor, New York, NY, 10032, USA. Electronic address:

Bullous diseases may be rare; however, this does not preclude the clinician from being familiar with their manifestations and treatment. After ruling out infection, genetically inherited blistering diseases are more likely to be the cause of blistering or erosions in the neonatal period, whereas immunobullous diseases are more common in adults. Published literature on immunobullous disorders reflects information gleaned from case reports and open-label case series; prospective studies and evidence-based treatments are limited. Read More

Nan Fang Yi Ke Da Xue Xue Bao 2015 Aug;35(9):1349-51

Department of Dermatology, Second Affiliated Hospital, Xi'an Jiaotong University College of Medicine, Xi'an 710004, China.E-mail:

Objevtive: To investigate the expression of transient receptor potential lvanilloidreceptor 4 (TRPV4) protein in pemphigus vulgaris (PV), bullous pemphigoid (BP), dermatitis herpetiformis (DH), and epidermolysis bullosa acquisita (EBA), and explore the role of TRPV4 in the pathogenesis of these diseases.

Methods: TRPV4 protein in normal skin tissues and lesions of PV, BP, DH, and EBA were detected with immunohistochemistry.

Results: The positivity rate of TRPV4 protein expression was 61. Read More

Pediatr Dermatol 2015 Nov-Dec;32(6):845-52. Epub 2015 Sep 22.

Division of Dermatology, National University Hospital, Singapore, Singapore.

Background: Autoimmune blistering diseases (AIBDs) are rare in children and their prevalence in Singapore is unclear. We aimed to investigate the clinical and immunopathologic characteristics of children diagnosed with AIBDs in Singapore.

Materials And Methods: The clinical and histology databases at the National Skin Centre in Singapore were searched to identify patients younger than 18 years old diagnosed with an AIBD from January 1, 1998, through December 31, 2012. Read More

Pediatr Dermatol 2015 Nov-Dec;32(6):e234-7. Epub 2015 Jul 27.

Division of Pediatric Dermatology, University of Minnesota, Minneapolis, Minnesota.

Pemphigus herpetiformis (PH) is characterized by grouped vesicular or papular pruritic lesions with histologic and immunopathologic features of pemphigus. PH can manifest at any age, and paraneoplastic cases have been reported. We describe a healthy boy born with acral crateriform erosions of the hands and feet whose 35-year-old mother had similar lesions. Read More

Indian Dermatol Online J 2015 May-Jun;6(3):172-80

Department of Dermatology, M. S. Ramaiah Medical College and Hospital, Bengaluru, Karnataka, India.

Background: Direct immunofluorescence (DIF) test for tissue-bound autoantibodies, has been found to be of value in the diagnosis of several dermatological disorders. The location and pattern of deposition of immunoreactants helps in classifying various immune-mediated diseases.

Aims And Objectives: The aim of this study was to analyze the concordance between the clinical, histopathological and DIF diagnosis in bullous and nonbullous lesions of the skin, and thus determine the impact of immunofluorescence on diagnosis. Read More

An Bras Dermatol 2015 Mar-Apr;90(2):190-4

Universidade Estadual Paulista "Júlio de Mesquita Filho", Botucatu, SP, Brazil.

Background: Autoimmune bullous dermatoses are complex diseases triggered by autoantibodies action against epidermal antigens or the dermoepidermal junction. Blisters and vesicles that evolve with erosion areas characterize them. Although rare, they present high morbidity, affecting the quality of life of patients. Read More

Br J Dermatol 2015 Jul 28;173(1):59-68. Epub 2015 May 28.

Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, 67 Asahimachi, Kurume, Fukuoka, 830-0011, Japan.

Background: Despite the established pathogenic role of anti-desmoglein (Dsg) antibodies in classical pemphigus, the significance of autoantibodies to another desmosomal cadherin, desmocollin (Dsc) is at present unknown. No consistent immunoassay for immunoglobulin (Ig) G autoantibodies to Dscs has been developed.

Objectives: The aim of this study was to develop reliable assays to detect anti-Dsc autoantibodies. Read More

Int J Dermatol 2015 Sep 20;54(9):1014-22. Epub 2015 Jan 20.

Department of Medicine, University of Toronto, Toronto, Ontario, Canada.

Background: Pemphigus herpetiformis (PH) is a rare subtype of pemphigus that presents challenges in diagnosis.

Objective: To review the presentation, diagnosis, and management of PH.

Methods: We reviewed the charts of all patients diagnosed and treated for PH in an immunobullous referral center between September 2007 and June 2013. Read More

J Eur Acad Dermatol Venereol 2016 Mar 29;30(3):540-2. Epub 2014 Dec 29.

Department of Dermatology, Baskent University Faculty of Medicine, Ankara Hospitals, Adana, Turkey.

An Bras Dermatol 2014 Nov-Dec;89(6):885-9

Universidade de São Paulo, São Paulo, SP, Brazil.

Background: Immunofluorescence testing is an important tool for diagnosing blistering diseases.

Objective: To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases.

Methods: We retrospectively analyzed immunofluorescence results encompassing a 10-year period. Read More

Ann Dermatol Venereol 2014 Oct 2;141(10):646-7. Epub 2014 Jun 2.

Service de dermatologie, hôpital Farhat-Hached, avenue Ibn El Jazzar, 4000 Sousse, Tunisie.

Chin Med J (Engl) 2014 ;127(19):3514

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

Br J Dermatol 2015 Apr 25;172(4):1144-6. Epub 2015 Feb 25.

Department of Dermatology and Cutaneous Biology Research Institute, Gangnam Severance Hospital, Yonsei University College of Medicine, Eonjuro 712, Gangnam-gu, 135-720, Seoul, Korea.

Indian J Dermatol 2014 Jul;59(4):364-8

Department of Pathology, Padmashree Dr. D.Y. Patil Medical College, Pimpri, Pune, India.

Background: Autoimmune blistering diseases are a group of bullous disorders characterized by pathogenic antibodies directed at the target antigens, which are components of the desmosomes or adhesion complex at the dermoepidermal junction. Direct immunofluorescence (DIF) is invaluable in the diagnosis of these lesions.

Aim: The aim of this study was to evaluate the sensitivity of DIF in immunobullous dermatoses and to study the pattern of DIF. Read More