Molecular Biological Aspects of Acquired Bullous Diseases

Dabelsteen, Erik

doi:10.1177/10454411980090020201

Cited by 36 publications

(30 citation statements)

References 149 publications

(159 reference statements)

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“…In recent years detailed characterization of the various target antigens have been made and subsets of pemphigoid subsequently identified. [34][35][36] Mucous membrane pemphigoid chiefly affects the oral cavity, larynx, oesophagus and ocular membranes but rarely the skin. Females in their sixth decade tend to be afflicted more commonly but males and patients in other age groups can be affected as well.…”

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

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Desquamative gingivitis: A sign of mucocutaneous disorders‐a review

Robinson

Wray

2003

Australian Dental Journal

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Desquamative gingivitis is a clinical term to describe red, painful, glazed and friable gingivae which may be a manifestation of some mucocutaneous conditions such as lichen planus or the vesiculobullous disorders. It is important to be aware of this rare clinical entity so as to distinguish desquamative gingivitis from plaque induced gingivitis which is an extremely common condition, easily recognized and treated daily by the dental practitioner. This article gives an overview of desquamative gingivitis, its presentation, the possible causes, diagnosis and treatment. Early recognition of these lesions may prevent delayed diagnosis and inappropriate treatment of potentially serious dermatological diseases.

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Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

“…The intercellular adhesion between the spinous cells is disrupted resulting in bulla formation immediately suprabasal. 33,34,48 In pemphigus foliaceus, which predominantly affects skin, autoantibodies are produced against desmoglein 1.…”

Section: Pemphigusmentioning

confidence: 99%

Desquamative gingivitis: A sign of mucocutaneous disorders‐a review

Robinson

Wray

2003

Australian Dental Journal

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“…Acomete pele e mucosas e caracteriza-se pela presença de auto-anticorpos contra proteínas desmossômicas encontrado nas junções epiteliais dos tecidos de revestimento 1 . A faixa etária mais acometida encontra-se entre a 5 a e 6 a décadas de vida, sendo menos freqüente após os 70 anos e em crianças 2,3 .…”

Section: Introdução Introdução Introdução Introdução Introduçãounclassified

Acometimento oral no pênfigo vulgar

Miziara

Filho

Ribeiro

et al. 2003

Rev. Bras. Otorrinolaringol.

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O Pênfigo Vulgar (PV) é uma doença vesicobolhosa caracterizada pela presença de auto-anticorpos contra moléculas de adesão intraepidérmicas. OBJETIVO: Identificar as características do PV em cada sexo, formas de apresentação, idade de acometimento, métodos de diagnóstico e tratamento utilizado. FORMA DE ESTUDO: coorte histórica. CASUÍSTICA E MÉTODO: Pacientes com PV acompanhados no Ambulatório de Otorrinolaringologia do HC-FMUSP entre 1990 e 2001. RESULTADOS: Dos 23 pacientes, 91,3% foram mulheres e 8,7% homens (p<0,01), relação de 9:1. A idade ao diagnóstico variou entre 26 e 80 anos, com média de 53 ± 18,4 anos. Nos homens, o PV manifestou-se 24,6 ± 5,9 anos mais precocemente do que nas mulheres (p=0,026). Os principais achados ao exame foram lesões exulceradas na mucosa oral, semelhantes a aftas. Biópsia foi utilizada em 95% dos casos, imunofluorescência direta em 17,4% e indireta em 8,7%. A prednisona foi a opção terapêutica em 78% dos pacientes, enquanto o deflazacort foi usado em 22%. A dapsona foi associada em 4 casos. Obtivemos controle da doença em 74% dos casos e perda do seguimento após melhora parcial em 13% dos pacientes. Observamos maior dificuldade no controle da doença no sexo masculino (p=0,04). CONCLUSÕES: O sexo feminino prevaleceu sobre o masculino. A idade ao diagnóstico foi maior nas mulheres. O tratamento de escolha foi a prednisona. O PV foi mais facilmente controlado nas mulheres.

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“…The pemphigus diseases are autoimmune blistering disorders in which separation of the suprabasal layers is found in conjunction with circulating desmoglein autoantibodies which label the cell surfaces of keratinocytes by direct immunofluorescence (reviewed in Amagai, 1996;Dabelsteen, 1998). The pemphigus diseases are frequently life-threatening, but now can be treated with corticosteroids.…”

Section: (4) Trichohyalinmentioning

confidence: 99%

Epithelial Structural Proteins of the Skin and Oral Cavity: Function in Health and Disease

Presland

Dale

2000

Critical Reviews in Oral Biology & Medicine

349

328

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Epithelial tissues function to protect the organism from physical, chemical, and microbial damage and are essential for survival. To perform this role, epithelial keratinocytes undergo a well-defined differentiation program that results in the expression of structural proteins which maintain the integrity of epithelial tissues and function as a protective barrier. This review focuses on structural proteins of the epidermis and oral mucosa. Keratin proteins comprise the predominant cytoskeletal component of these epithelia. Keratin filaments are attached to the plasma membrane via desmosomes, and together these structural components form a threedimensional array within the cytoplasm of epithelial cells and tissues. Desmosomes contain two types of transmembrane proteins, the desmogleins and desmocollins, that are members of the cadherin family. The desmosomal cadherins are linked to the keratin cytoskeleton via several cytoplasmic plaque proteins, including desmoplakin and plakoglobin (-y-catenin). Epidermal and oral keratinocytes express additional differentiation markers, including filaggrin and trichohyalin, that associate with the keratin cytoskeleton during terminal differentiation, and proteins such as loricrin, small proline-rich proteins, and involucrin, that are cross-linked into the cornified envelope by transglutaminase enzymes. The importance of these cellular structures is highlighted by the large numbers of genetic and acquired (autoimmune) human disorders that involve mutations in, or autoantibodies to, keratins and desmosomal and cornified envelope proteins. While much progress has been made in the identification of the structural proteins and enzymes involved in epithelial differentiation, regulation of this process is less clear. Both calcium and retinoids influence epithelial differentiation by altering the transcription of target genes and by regulating activity of enzymes critical in epithelial differentiation, such as transglutaminases proteinases, and protein kinases. These studies have furthered our understanding of how epithelial tissue and cell integrity is maintained and provide a basis for the future treatment of skin and oral disorders by gene therapy and other novel therapeutics.

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Molecular Biological Aspects of Acquired Bullous Diseases

Cited by 36 publications

References 149 publications

Desquamative gingivitis: A sign of mucocutaneous disorders‐a review

Desquamative gingivitis: A sign of mucocutaneous disorders‐a review

Acometimento oral no pênfigo vulgar

Epithelial Structural Proteins of the Skin and Oral Cavity: Function in Health and Disease

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