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

J Periodontol 2009 Nov;80(11):1765-73

Department of Periodontics, Baylor College of Dentistry, The Texas A&M Health Science Center, Dallas, TX 75246, USA.

Background: Mucous membrane pemphigoid (MMP) is a heterogeneous group of blistering autoimmune disorders of unknown etiology. Intraoral manifestations of MMP feature the formation of vesiculobullous lesions that eventually rupture, leading to pseudomembrane-covered, irregularly-shaped ulcerations. The presence of these often painful oral lesions may hinder oral hygiene efforts resulting in increased plaque accumulation and may increase the risk of developing periodontal disease. Read More

Minerva Stomatol 2009 Oct;58(10):501-18

Oral Medicine Unit, Department of Odontostomatologica and Maxillo-facial Science, School of Medicine and Surgery, Federico II University, Naples, Italy.

Pemphigus is a group of potentially life-threatening autoimmune diseases characterized by cutaneous and/or mucosal blistering, due to the presence circulating IgG antibodies directed against desmoglein 1 and 3 (Dsg 1 and 3). Differences in the particular distribution of these result in different clinical manifestations of the disease. The most common variant is pemphigus vulgaris (PV). Read More

J Eur Acad Dermatol Venereol 2008 Dec;22(12):1478-96

Aristotle University Medical School, Thessaloniki, Greece.

Pemphigus refers to a group of potentially life-threatening autoimmune diseases of the skin and mucous membranes, characterized by the formation of blisters and erosions of the skin. An autoimmune process, directed against keratinocyte desmosomal cadherins, interferes with the adhesive function of these molecules. This results in the separation of keratinocytes and clinical manifestation of blistering. Read More

Kathmandu Univ Med J (KUMJ) 2007 Oct-Dec;5(4):541-5

Department of Oral Medicine and Radiology, Manipal College of Dental Sciences, Mangalore, India.

Pemphigus vulgaris is an autoimmune blistering disease affecting the mucous membrane and skin. Ulcers, vesicles, bulla, erosions are the common manifestations of the disease. It is uncommon to find multiple pustular lesions in the oral cavity. Read More

Ophthalmologe 2008 Mar;105(3):285-97; quiz 298

Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Julius-Maximilians-Universität Würzburg, Würzburg, Deutschland.

Mucous membrane pemphigoid is a subepidermal blistering autoimmune disorder characterized by predominant involvement of mucous membranes and the presence of autoantibodies against proteins of the dermal-epidermal junction. Lesions most frequently develop in the oral cavity followed, in descending order of frequency, by conjunctiva, nasopharynx, the anogenital region, skin, larynx, and oesophagus. When the lesions are restricted to the conjunctiva, the term ocular pemphigoid may be applied. Read More

J Am Acad Dermatol 2007 May;56(5 Suppl):S94-7

Department of Dermatology, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan.

We report the case of a 55-year-old female with bullous pemphigoid (BP) who was positive for anti-BP180 and anti-laminin 5 antibodies after development of graft-vs-host disease (GVHD) caused by a bone marrow transplant. She had tense blisters on her trunk and extremities. Histologic examination showed a subepidermal blister and marked lymphocytic infiltration, especially eosinophils. Read More

Lancet 2005 Jul 2-8;366(9479):61-73

The Ronald O Perelman Department of Dermatology, New York University School of Medicine, 560 1st Avenue, New York, NY 10016, USA.

Pemphigus is a rare autoimmune disease that results in blistering of the skin and oral cavity. It is caused by autoantibodies directed against cell-surface antigens on keratinocytes, which when targeted lose their cellular adhesion properties and separate from one another to form blisters within the epidermis. Differences in the particular antigens targeted by the antibodies and in the distribution of these antigens in the different regions of the body and in the separate layers of the epidermis result in different clinical manifestations of the disease. Read More

Arch Otolaryngol Head Neck Surg 2004 Apr;130(4):469-72

Department of Oral Surgery, Oral Medicine, and Oral Pathology, Dublin Dental School and Hospital, Ireland.

Linear IgA disease is a subepidermal blistering disorder of unknown cause. Along with dermatologic blistering, other manifestations include oral ulceration and desquamative gingivitis. We present a case of linear IgA disease that manifested simply as oropharyngeal ulceration and desquamative gingivitis, resulting in considerable delay in diagnosis and treatment. Read More

Med Oral 2004 Jan-Feb;9(1):39-44

Medicina Bucal, Departamento de Estomatología, Universidad del País Vasco, España.

The Linear IgA deposit related disease or Linear IgA disease (LAD) is a chronic, uncommon and autoimmunological mucocutaneous disease, characterised by linear IgA deposits along the basement membrane zone. In mainly cases, moreover cutaneous lesions, there are oral mucosal and other mucosal lesions. There are also, some cases published of Linear IgA disease limited to oral mucosa. Read More

J Dermatol 2003 Nov;30(11):782-8

Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea.

Pemphigus, a rare, chronic blistering disease of the skin and mucous membranes with severe morbidity and occasional mortality, is the most common autoimmune bullous disease in Korea. The purpose of this study was to evaluate the clinical features and propose a treatment strategy for patients with pemphigus. A retrospective analysis was conducted of 51 pemphigus patients seen between 1993 and 2001. Read More

J Periodontol 2003 Jun;74(6):879-82

Buccal Medicine, University of the Basque Country (EHU), Vizcaya, Spain.

Linear IgA disease (LAD) is a chronic, subepithelial blistering disease that is associated with the presence of linear deposits of IgA along the basement membrane zone. Etiopathogenic aspects of LAD are only partially known. LAD is clinically characterized by vesiculobullous skin and mucous lesions. Read More

Pediatrics 2003 Jul;112(1 Pt 1):183-5

Department of Paediatrics, Hospital for Sick Children, University of Toronto, Toronto, Canada M5G 1X8.

Pemphigus vulgaris is a potentially fatal autoimmune blistering disease that is rare in childhood. We report 2 recent cases seen contemporaneously in adolescents who presented with chronic oral mucosal lesions and conjunctivitis. The previously unemphasized ocular manifestations are described and the importance of a biopsy in establishing the diagnosis in instances of unexplained chronic mucositis is stressed. Read More

Transfus Apher Sci 2003 Feb;28(1):13-8

Servizio Immunotrasfusionale, Azienda Ospedaliera S Maria degli Angeli, Via Montereale 24, 33170 Pordenone, Italy.

Blistering dermatitises are characterized by the presence of blisters that begin owing to acantholysis (intraepidermic blister) such as pemphigus vulgaris (PV) or owing to dermoepidermic detachment (subepidermic blister) such as bullous pemphigoid (BP). Both diseases are autoimmune pathologies characterized by the presence of autoantibodies against specific adhesion molecules of the skin and mucous membranes. PV, in which oral lesions are always present, has a progressive course that, if the disease is not treated, nearly always brings to death from sepsis within a few years. Read More

Crit Rev Oral Biol Med 2002 ;13(5):397-408

Department of Oral Medicine, Eastman Dental Institute for Oral Health Care Sciences, University College London, University of London, 256 Gray's Inn Road, London WC1X 8LD, UK.

Pemphigus is a group of potentially life-threatening diseases characterized by cutaneous and mucosal blistering. There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles. Certain ethnic groups, such as Ashkenazi Jews and those of Mediterranean origin, are especially liable to pemphigus. Read More

Ann N Y Acad Sci 2002 Jun;966:429-40

UCLA School of Dentistry, and Dental Research Institute, Los Angeles, California 90095, USA.

Rheumatoid arthritis involves psychoneuroendocrine-immunopathological comorbidities. In the stoma, patients with rheumatoid arthritis frequently show signs of periondontal disease consequent to elevated levels of crevicular proinflammatory cytokines. It is not clear whether rheumatoid arthritis may manifest in association with immunopathological manifestations of the oral soft mucosa. Read More

Ophthalmologe 2000 Feb;97(2):113-20

Augenklinik der Ludwig-Maximilians-Universität, München.

Unlabelled: Cicatricial pemphigoid (CP) is a rare autoimmune disease characterized by subepidermal blistering and progressive cicatrization affecting the skin and mucosa. Ocular involvement occurs in approximately 70% of the patients.

Methods: The course of the disease, complications and putative risk factors in patients with ocular cicatricial pemphigoid (OCP) treated at the Departments of Ophthalmology and Dermatology were analyzed retrospectively from 1986 to 1998. Read More

Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997 Nov;84(5):517-34

Department of Periodontics, New York University College of Dentistry, NY 10010, USA.

This review will describe adult onset mucocutaneous/autoimmune diseases that involve defects in cell-to-cell, cell-to-matrix, or cell-to-basement membrane adhesion. Included in this group are pemphigus, cicatricial pemphigoid, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, and bullous systemic lupus erythematous. Detection and treatment of blistering disorders that manifest early in the oral cavity may prevent widespread involvement of skin. Read More

Clin Exp Dermatol 1996 Jan;21(1):23-7

Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.

The relationship between clinical findings and antigen profiles in 100 bullous pemphigoid (BP) patients has been investigated. The patients were divided into four groups based upon the results of immunoblot analysis, namely patients whose sera detected the 230-kDa BP antigen (BP230) and the 180-kDa BP antigen (BP180), those recognizing either BP230 or BP180 alone, and those recognizing neither antigen. Analysis by the chi-squared test showed predominant occurrence of oral (P < 0. Read More

Proc Natl Acad Sci U S A 1994 Aug;91(16):7747-51

Division of Immunogenetics, Dana-Farber Cancer Institute, Boston, MA 02115.

Cicatricial pemphigoid (CP) is a chronic autoimmune blistering disease affecting multiple mucous membranes derived from stratified squamous epithelium and occasionally the skin. CP has a wide spectrum of disease manifestations. Patients with oral pemphigoid (OP) have a benign self-limited disease in which pathological changes are restricted to the oral mucosa. Read More