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'Oral Manifestations of Autoimmune Blistering Diseases' (36)


Oct
2017

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).

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Dec
2017

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.

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May
2017

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.

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May
2017

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.

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May
2017

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.

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Mar
2017

Desmosomes in disease: a guide for clinicians.

Oral Dis 2017 Mar 19;23(2):157-167. Epub 2016 Jul 19.
A Celentano, N Cirillo
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.

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Jul
2016

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.

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Oct
2015

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.

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Dec
1969

Pemphigus vulgaris presented with cheilitis.

Case Rep Dermatol Med 2014 25;2014:147197. Epub 2014 Sep 25.
Zaheer Abbas, Zahra Safaie Naraghi, Elham Behrangi
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.

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Sep
2014

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.
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.

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Nov
2013

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.

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Nov
2012

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.

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Aug
2011

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.

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Apr
2011

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.

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Sep
2011

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.

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Feb
2011

Vesiculobullous eruptions of the oral cavity.

Otolaryngol Clin North Am 2011 Feb;44(1):133-60, vi
Sherif Said, Loren Golitz
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.

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Aug
2009

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.

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Nov
2009

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.

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Oct
2009

Oral pemphigus.

Minerva Stomatol 2009 Oct;58(10):501-18
M D Mignogna, G Fortuna, S Leuci
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).

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Dec
2008

Pemphigus.

J Eur Acad Dermatol Venereol 2008 Dec;22(12):1478-96
D Ioannides, E Lazaridou, D Rigopoulos
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.

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Dec
2008

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.

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Mar
2008

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.

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May
2007

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.

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May
2007

Pemphigus.

Lancet 2005 Jul 2-8;366(9479):61-73
Jean-Claude Bystryn, Jennifer L Rudolph
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.

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Apr
2004

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.

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Apr
2004

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.

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Nov
2003

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.

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Jun
2003

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.

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Jul
2003

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.

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Feb
2003

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.

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Dec
1969

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.

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Jun
2002

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.

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Feb
2000

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.
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.

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Nov
1997

Mucocutaneous features of autoimmune blistering diseases.

Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997 Nov;84(5):517-34
M A Weinberg, M S Insler, R B Campen
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.

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Jan
1996

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.

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Aug
1994

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.

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