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4.
Aust Dent J ; 60(1): 18-23, 2015 Mar.
Article in English | MEDLINE | ID: mdl-25721275

ABSTRACT

BACKGROUND: Gingival lesions in patients with dystrophic epidermolysis bullosa (DEB) are a common manifestation. However, their clinical features, frequency and severity are currently unknown. METHODS: Forty-five DEB patients were assessed by an oral medicine specialist, who analysed the presence/absence of four clinical signs (erythema, erosion/ulcer, atrophy, blister) on free and attached gingiva, using the Epidermolysis Bullosa Oropharyngeal Severity score. RESULTS: Twenty-eight (62.2%) out of 45 DEB patients showed different types of gingival lesions, whose presence/absence and total frequency/distribution were not significantly different between males and females (p=0.087 and p=0.091, respectively). Erythema was the most prevalent lesion (66.2%) and the recessive DEB severe generalized (RDEB-sev gen) reached the highest median disease activity score. A significant correlation was observed between the DEB subtypes and the disease activity median score (p<0.001), but not between age and total disease activity score in each group of DEB (p>0.05). Lastly, logistic regression showed that only gender (p=0.031) and RDEB-sev gen (p=0.001) were risks factors for the presence of gingival lesions. CONCLUSIONS: Gingival lesions in DEB patients are a relatively common entity and may have multiple clinical aspects, emphasizing the need for thorough attention and awareness among dentists.


Subject(s)
Epidermolysis Bullosa Dystrophica/pathology , Gingival Diseases/pathology , Adolescent , Adult , Blister/pathology , Child , Child, Preschool , Cross-Sectional Studies , Epidermolysis Bullosa Dystrophica/classification , Erythema/pathology , Female , Gingival Diseases/classification , Humans , Infant , Male , Middle Aged , Oral Ulcer/pathology , Periodontal Atrophy/pathology , Prevalence , Risk Factors , Sex Factors , Young Adult
5.
Actas Dermosifiliogr ; 101(8): 673-82, 2010 Oct.
Article in Spanish | MEDLINE | ID: mdl-20965010

ABSTRACT

The tools for diagnosis of epidermolysis bullosa have advanced greatly since Hintner's group introduced antigen mapping as a diagnostic test for this family of genodermatoses. Monoclonal or polyclonal antibodies raised against some of the specific proteins found in the epidermis and basement membrane of the epidermis have allowed 4 types of epidermolysis bullosa de be identified and all variants to be classified. When a newborn baby presents with blisters, many conditions are implicated in the differential diagnosis. Examination under an optical microscope can suggest epidermolysis bullosa, but immunofluorescence mapping and electron microscopy are required for confirmation of the diagnosis and further classification of congenital epidermolysis bullosa. This article explains the importance of immunofluorescence antigen mapping and describes the methods employed for classification and subclassification of epidermolysis bullosa.


Subject(s)
Epidermolysis Bullosa/diagnosis , Fluorescent Antibody Technique, Direct , Basement Membrane/immunology , Biopsy , Diagnosis, Differential , Epidermis/immunology , Epidermis/ultrastructure , Epidermolysis Bullosa/classification , Epidermolysis Bullosa/immunology , Epidermolysis Bullosa/pathology , Epidermolysis Bullosa Dystrophica/diagnosis , Epidermolysis Bullosa Dystrophica/immunology , Epidermolysis Bullosa Dystrophica/pathology , Epidermolysis Bullosa Simplex/diagnosis , Epidermolysis Bullosa Simplex/immunology , Epidermolysis Bullosa Simplex/pathology , Epidermolysis Bullosa, Junctional/diagnosis , Epidermolysis Bullosa, Junctional/immunology , Epidermolysis Bullosa, Junctional/pathology , Humans , Infant, Newborn , Microscopy, Fluorescence , Specimen Handling
6.
Actas dermo-sifiliogr. (Ed. impr.) ; 101(8): 673-682, oct. 2010. ilus, tab
Article in Spanish | IBECS | ID: ibc-87821

ABSTRACT

Las herramientas para el diagnóstico en las epidermólisis ampollosas (EA) han tenido un gran avance desde que Hintner et al, introdujeron el mapeo antigénico como prueba diagnóstica en este grupo de genodermatosis. La utilización de anticuerpos monoclonales/policlonales dirigidos contra algunas de las proteínas específicas que conforman la epidermis y la membrana basal epidérmica, han servido para clasificar los 4 tipos de epidermólisis ampollosa y subclasificar todas sus variantes. Ante la presencia de un recién nacido con ampollas surgen diagnósticos diferenciales múltiples, en donde la microscopia de luz orienta el diagnostico de epidermólisis ampollosa. Sin embargo, el mapeo por inmunofluorescencia y la microscopia electrónica permiten confirmar y clasificar a las epidermólisis ampollosas congénitas. En este artículo, se explica la importancia y metodología para desarrollar la técnica de mapeo antigénico por inmunofluorescencia, con el propósito de clasificar y subclasificar las epidermólisis ampollosas (AU)


The tools for diagnosis of epidermolysis bullosa have advanced greatly since Hintner's group introduced antigen mapping as a diagnostic test for this family of genodermatoses. Monoclonal or polyclonal antibodies raised against some of the specific proteins found in the epidermis and basement membrane of the epidermis have allowed 4 types of epidermolysis bullosa de be identified and all variants to be classified. When a newborn baby presents with blisters, many conditions are implicated in the differential diagnosis. Examination under an optical microscope can suggest epidermolysis bullosa, but immunofluorescence mapping and electron microscopy are required for confirmation of the diagnosis and further classification of congenital epidermolysis bullosa. This article explains the importance of immunofluorescence antigen mapping and describes the methods employed for classification and subclassification of epidermolysis bullosa (AU)


Subject(s)
Humans , Male , Female , Fluorescent Antibody Technique, Direct/instrumentation , Fluorescent Antibody Technique, Direct/methods , Fluorescent Antibody Technique, Direct , Epidermolysis Bullosa/diagnosis , Epidermolysis Bullosa/pathology , Epidermolysis Bullosa/therapy , Biopsy/instrumentation , Biopsy/methods , Antibodies/analysis , Antibodies/immunology , Collagen/analysis , Epidermolysis Bullosa Simplex/diagnosis , Epidermolysis Bullosa Simplex/pathology , Epidermolysis Bullosa Dystrophica/diagnosis , Epidermolysis Bullosa Dystrophica/pathology
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