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Apoptosis 2022 06 20;27(5-6):322-328. Epub 2022 Apr 20.

Department of Oral Pathology and Microbiology, Saveetha Dental College and Hospitals, Chennai, Tamil Nadu, India.

Pemphigus Vulgaris (PV) is a severe autoimmune disease characterized by supra-basal blisters in the skin and mucous membranes of a wide range of mammals, including humans. It not only affects the skin but also has severe oral manifestations. It has been stated that auto-antibodies are produced, for unknown reasons, which are directed against desmogleins present on the epithelium and thus leads to acantholysis and intraepithelial blistering. Read More

Cureus 2022 Jan 30;14(1):e21739. Epub 2022 Jan 30.

Dermatology, Elias Emergency University Hospital, Bucharest, ROU.

Pemphigus defines a group of rare autoimmune blistering diseases that affect the skin and mucous membranes, with pemphigus vulgaris being the most common form that has increased morbidity and mortality in the absence of an early diagnosis and treatment. We report the case of a 24-year-old male with an atypical form of pemphigus vulgaris with cutaneous onset and subsequent involvement of the oral cavity. The management of the patient initially consisted of long-term systemic corticosteroid therapy. Read More

Front Immunol 2021 7;12:812627. Epub 2022 Feb 7.

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

Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease characterized by autoantibodies against the basal membrane zone of skin and surface-close epithelia and predominant mucosal lesions. The oral cavity and conjunctivae are most frequently affected, albeit clinical manifestations can also occur on the skin. MMP-associated lesions outside the oral cavity typically lead to scarring. Read More

Int J Dermatol 2022 Jan 20. Epub 2022 Jan 20.

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

Background: Brunsting-Perry pemphigoid (BPP) is a rare, autoimmune bullous skin disorder classified within the spectrum of mucous membrane pemphigoid (MMP).

Materials And Methods: An a priori protocol was designed based on PRISMA guidelines. PubMed and Scopus databases were searched for English-language articles concerning BPP published between 1950 and July 2021. Read More

Case Rep Dent 2021 25;2021:7792360. Epub 2021 Oct 25.

Department of Dental Medicine, Sahloul Hospital (Sousse), Dental Faculty of Monastir, University of Monastir, Tunisia.

The place of pemphigus vulgaris (PV) among autoimmune bullous diseases (AIBD) is well established. It is an acquired chronic, autoimmune, vesiculobullous disease in which IgG antibodies target desmosomal proteins to produce intraepithelial mucocutaneous blistering. The diagnosis is often challenging for the clinicians. Read More

Contemp Clin Dent 2020 Jul-Sep;11(3):277-279. Epub 2020 Nov 26.

Department of Periodontics, H. P. Government Dental College and Hospital, Shimla, Himachal Pradesh, India.

Pemphigus vulgaris (PV) is an autoimmune mucocutaneous disease characterized by epithelial blistering affecting the mucosal/cutaneous surfaces. This case report demonstrates the oral manifestations of PV in the uncommon locations such as the attached, marginal, and interdental gingiva with no involvement of oral mucosa, tongue, palate, or buccal mucosa. Read More

Eur Arch Otorhinolaryngol 2020 Mar 24;277(3):939-945. Epub 2020 Jan 24.

Department of Otolaryngology and Head and Neck Surgery, Sheba Medical Center, 52621, Tel Hashomer, Israel.

Introduction: Mucous membrane pemphigoid (MMP) refers to a heterogeneous group of rare, chronic, inflammatory, mucous membrane-dominated, sub-epithelial blistering diseases that manifest with a varying constellation of oral, ocular, cutaneous, genital, nasopharyngeal, esophageal, and laryngeal lesions. MMP can progress to scarring in affected areas, which may lead to devastating complications including ocular involvement leading to blindness as well as laryngeal involvement leading to airway obstruction.

Materials And Methods: A retrospective chart review was conducted for patients that were followed in two tertiary academic centers between 2009 through 2017 for upper aerodigestive tract manifestations of MMP. Read More

J Family Med Prim Care 2019 Dec 10;8(12):4036-4038. Epub 2019 Dec 10.

Department of Oral Medicine and Radiology, Sharad Pawar Dental College, DMIMS (Deemed to be University), Wardha, Mahrashtra, India.

Pemphigus Vulgaris is an autoimmune disease that causes severe blistering of the skin and mucous membrane which are fragile and breaks away leaving erosions that are extremely painful. Here we report a case of a patient suffering from pemphigus vulgaris with typical oral manifestations. We have reported the effect of treatment after two consecutive recalls at an interval of 1 week where the patient got 100% relief from her previous signs and symptoms. Read More

BMC Gastroenterol 2019 Jun 14;19(1):92. Epub 2019 Jun 14.

Department of Organ transplantation, Sichuan Academy of Medical Science and Sichuan Provincial People's Hospital, 32 West Second Section, First Ring Road, Chengdu, 610072, Sichuan, China.

Background: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm that originates from follicular dendritic cells in lymphoid tissue while paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. Pancreatic FDCS associated with PNP and myasthenia gravis (MG) is even rarer and highly malignant. We present the clinical data, pathological materials and computed tomography (CT) features of a rare case of this disease. Read More

Stomatologiia (Mosk) 2019 ;98(2):71-75

Central Research Institute of Dentistry and Maxillofacial surgery, Moscow, Russia.

OBJECTIVE Based on the data available in modern literature, to conduct a study on the epidemiology, etiology and pathogenesis of bullous lesions of the oral mucosa (pemphigus vulgaris, pemphigoid bullosa, lichen ruber planus).

Material And Methods: The article is based on the study of materials of foreign and domestic research databases Pubmed and Elibrary. Used literature data over the past 15 years. Read More

Acta Dermatovenerol Croat 2018 Oct;26(3):273-275

Baoqi Yang, MD, Department of Dermatology Shandong Provincial Institute of Dermatology and Venereology Shandong Academy of Medical Sciences 27397 Jingshi Road, Jinan, Shandong, 250022, China;

Dear Editor, Dermatitis herpetiformis (DH) is a chronic, polymorphic, pruritic autoimmune blistering skin disease characterized by subepidermal blisters, neutrophilic microabscesses, and granular IgA deposition within the dermal papillae. DH is classified as a cutaneous manifestation of coeliac disease, a type of gluten-sensitive enteropathy (1). The treatment of DH includes dapsone and a gluten-free diet (GFD). Read More

Australas J Dermatol 2019 May 14;60(2):e105-e108. Epub 2018 Sep 14.

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

Background/objectives: The aim of this study was to describe a previously unreported association of oral pemphigus vulgaris with short-lived blood-filled painless blisters resembling angina bullosa haemorrhagica (ABH).

Methods: A cross-sectional study of consecutive patients with Pemphigus vulgaris. All patients were examined for the presence of ABH-like lesions, and demographic, clinical and histopathological data were collected. Read More

J Stomatol Oral Maxillofac Surg 2018 Dec 8;119(6):506-509. Epub 2018 Jun 8.

Department of oral and maxillofacial surgery, cliniques universitaires Saint-Luc, université catholique de Louvain, avenue Hippocrate 10, 1200 Woluwe-Saint-Lambert, Belgium.

Paraneoplastic pemphigus is a rare autoimmune blistering disease generally associated with malignancy. The clinical presentation consists typically of painful and diffuse erosive stomatitis that may be accompanied by polymorphic skin lesions and systemic involvement. Diagnosis is based on clinical manifestations and confirmed by histology and immunological testing. Read More

Acta Dermatovenerol Croat 2017 Oct;25(3):255-256

Sheng-Yiao Lin, No. 100, Tzyou 1st Road, Kaohsiung 807, Taiwan;

Dear Editor, Bullous pemphigoid (BP), a relatively common autoimmune blistering disease in the elderly, is characterized by large, tense bullae on urticarial, erythematous, or normal skin. However, atypical BP with polymorphic clinical presentations is rarely encountered, leading to misdiagnosis and delayed treatments (1). BP with lesions resembling erythema gyratum repens or figurate erythema has been regarded as a paraneoplastic phenomenon (1). Read More

J Dermatol 2018 Apr 4;45(4):472-474. Epub 2017 Dec 4.

Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.

Subepidermal autoimmune blistering disease including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, anti-laminin-γ1 pemphigoid, linear immunoglobulin A bullous disease and epidermolysis bullosa acquisita (EBA), are all characterized by direct immunofluorescence microscopy or immunoglobulin deposition on the basement membrane zone. Among them, EBA is a rare acquired subepidermal autoimmune blistering disease of the skin and mucous membranes reactive with type VII collagen, a major component of the epidermal basement membrane zone. Anti-laminin-332-type mucous membrane pemphigoid has pathogenic autoantibodies against laminin-332, which is a basement membrane heterotrimeric protein composed of α3, β3 and γ2 laminin chains. Read More

Nat Rev Dis Primers 2017 May 11;3:17026. Epub 2017 May 11.

Department of Dermatology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.

Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell-cell adhesion molecules found in desmosomes. Read More

J Oral Maxillofac Pathol 2016 Sep-Dec;20(3):549

Department of Oral Pathology, SRM Dental College, Chennai, Tamil Nadu, India.

Pemphigus vulgaris (PV) is a chronic, autoimmune, intraepidermal blistering disease of the skin and mucous membranes. The initial clinical manifestation is frequently the development of intraoral lesions, and later, the lesions involve the other mucous membranes and skin. The etiology of this disease still remains obscure although the presence of autoantibodies is consistent with an autoimmune disease. Read More

J Eur Acad Dermatol Venereol 2017 May 14;31(5):791-797. Epub 2016 Sep 14.

Department of Otorhinolaryngology, School of Medicine, University Clinic of Navarra, Navarra, Spain.

Autoimmune blistering diseases (AIBD) comprise several entities characterized by the presence of autoantibodies targeted against structural proteins either in desmosomes or in the dermoepidermal junction of polystratified squamous epithelium. Patients develop blisters, erosions in cutaneous surfaces or mucosas. Diagnosis is based on the characteristic mucocutaneous lesions, the typical findings on histological studies and direct immunofluorescence assays, and the presence of specific autoantibodies against the epidermal antigens. Read More

Oral Dis 2017 Mar 19;23(2):157-167. Epub 2016 Jul 19.

Melbourne Dental School and Oral Health CRC, University of Melbourne, Melbourne, Vic., Australia.

The large number of diseases occurring when desmosome constituents are impaired provides striking evidence for the key role of desmosomes in maintaining tissue integrity. A detailed understanding of the molecular alterations causing desmosomal dysfunction has, in turn, underpinned the development of novel diagnostic tools. This has salient clinical implications for dentists and oral medicine practitioners because the majority of desmosomal diseases affect the oral cavity. Read More

Spec Care Dentist 2016 Jul 27;36(4):223-30. Epub 2016 Jan 27.

Professor, Oral Medicine Department, Federal University of Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil.

Kindler syndrome is a rare genetic disorder showing some predominant clinical manifestations, for example, trauma-induced blisters, progressive poikiloderma, skin atrophy, and photosensitivity. Oral manifestations are not commonly described and can be often misdiagnosed. This report describes the case of a female patient diagnosed with Kindler syndrome showing the classical clinical features affecting the skin, in addition to oral lesions manifesting as keratotic plaques and ulcers affecting the buccal mucosa, floor of the mouth, alveolar ridge, hard palate, and soft palate. Read More

Autoimmun Rev 2015 Oct 24;14(10):930-51. Epub 2015 Jun 24.

Department of Dermatology, University of Freiburg, Hauptstrasse 7, 79104 Freiburg, Germany; BIOSS Centre for Biological Signalling Studies, Signalhaus Freiburg, Schänzlestr. 18, 79104 Freiburg, Germany. Electronic address:

A group of autoimmune diseases is characterised by autoantibodies against epithelial adhesion structures and/or tissue-tropic lymphocytes driving inflammatory processes resulting in specific pathology at the mucosal surfaces and the skin. The most frequent site of mucosal involvement in autoimmune diseases is the oral cavity. Broadly, these diseases include conditions affecting the cell-cell adhesion causing intra-epithelial blistering and those where autoantibodies or infiltration lymphocytes cause a loss of cell-matrix adhesion or interface inflammation. Read More

Case Rep Dermatol Med 2014 25;2014:147197. Epub 2014 Sep 25.

Department of Dermatology, Rasoul-e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.

Background. Pemphigus vulgaris is an autoimmune blistering disease affecting the mucous membrane and skin. In 50 to 70% of cases, the initial manifestations of pemphigus vulgaris are oral lesions which may be followed by skin lesions. Read More

Oral Health Dent Manag 2014 Sep;13(3):605-9

Department of Oral Surgery and Medicine, Dental Faculty, Istanbul University, Millet cad. 34093 Çapa, Istanbul, Turkey, Tel: +90-212-4142020, Ext: 30353; Fax: +90-212-4142564; e-mail:

Purpose: Pemphigus, defines a group of disorders in autoimmune etiology which could be life-threatening and clinical manifestations are mainly epithelial blistering affecting cutaneous and/or mucosal surfaces including oral mucosa. The aim of our study is to evaluate the clinical appearance of pemphigus with oral involvement by reported 15 pemphigus cases.

Material And Methods: This retrospective study of 15 cases of pemphigus obtained over a period of 7 years from 2006 to 2013 in Istanbul University, Dentistry Faculty, Oral Medicine and Surgery Department was designed. Read More

BMC Oral Health 2013 Nov 21;13:66. Epub 2013 Nov 21.

Background: Pemphigus is a rare group of life-threatening mucocutaneous autoimmune blistering diseases. Frequently, oral lesions precede the cutaneous ones. This study aimed to describe clinical and histological features of oral pemphigus lesions in patients aged 18 years and above, attending outpatient's facility of Khartoum Teaching Hospital - Dermatology Clinic, Sudan. Read More

Br J Dermatol 2012 Nov;167(5):1011-6

Department of Otorhinolaryngology, Unit of Autoimmune Blistering Skin Diseases, University Clinic of Navarra, School of Medicine, PO Box 4209, Pamplona 31080, Navarra, Spain.

Background: Pemphigus vulgaris (PV) is an autoimmune blistering skin disorder characterized by the presence of suprabasal acantholysis and autoantibodies against desmoglein 3. There are two different clinical forms: mucocutaneous (MCPV) and mucosal (MPV). However, little is known about PV lesions in oral, ear, nose and throat (OENT) areas produced by the very dynamic of the anatomical structures involved in the functions of the aerodigestive tract. Read More

Optom Vis Sci 2011 Aug;88(8):1010-3

The Eye Institute at Pennsylvania College of Optometry at Salus University, Philadelphia, Pennsylvania, USA.

Purpose: Pemphigus vulgaris (PV) is an autoimmune blistering disease that affects mucous membranes and the skin. Most commonly, the disease begins in the oral cavity and spreads to other areas including the conjunctiva and eyelids. Ocular involvement is rare and likely underdiagnosed with a frequency that is underestimated. Read More

Hautarzt 2011 Apr;62(4):262-4

Hautklinik des Universitätsklinikums Düsseldorf, Heinrich-Heine-Universität Düsseldorf, Moorenstr. 5, 40225 Düsseldorf, Deutschland.

Linear IgA bullous dermatosis is an acquired autoimmune subepidermal blistering disease, characterized by linear IgA deposits at the basement membrane zone. Described in both children and adults, it occurs as tense pruritic vesicles and bullae in a "cluster of jewels" configuration with central crusting on an inflammatory elevated base. It is typically located on the face, anogenital region and trunk. Read More

J Oral Pathol Med 2011 Sep 9;40(8):616-20. Epub 2011 Mar 9.

Department of Dermatology, King's College Hospital, London, UK.

Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering disease affecting the skin and mucosa and is associated with increased morbidity and mortality. Once the diagnosis is established, the main stay of treatment of PV is with systemic corticosteroids to control the disease and then to consolidate the management with other immunosuppressive agents. A small group of patients with severe pemphigus, however, remain relcalcitrant to both steroids and azathioprine, and disease is difficult to control. Read More

Otolaryngol Clin North Am 2011 Feb;44(1):133-60, vi

Department of Pathology, University of Colorado Denver, Aurora, CO 80045, USA.

The spectrum of vesiculobullous eruptions of the oral cavity is wide and rich, with different disease entities that encompass different etiologies, pathogenesis, clinical manifestations, treatment plans, and prognostic ends. Trying to present all these entities in a comprehensive fashion is challenging, but in this article, most of the important entities pertaining to this topic have been encompassed in a concise manner. Read More

Acta Otorhinolaryngol Ital 2009 Aug;29(4):222-5

Department of Otorhinolaryngology, S. Raffaele Hospital, Vita-Salute University, Via Olgettina 60, Milan, Italy.

Mucous membrane pemphigoid includes chronic autoimmune sub-epithelial blistering diseases that predominantly affect mucous membranes, with varying combinations of oral, ocular, cutaneous, genital, nasopharyngeal, oesophageal and laryngeal lesions. The case is reported of a man with multiple manifestations of mucous membrane pemphigoid. A 53-year-old male presented at our Department with a 4-year clinical history of diagnosed cicatricial pemphigoid. Read More