Bullous pemphigoid

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dc.contributor Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.author MIYAMOTO, Denise FMUSP-HC
SANTI, Claudia Giuli FMUSP-HC
AOKI, Valeria FMUSP-HC
MARUTA, Celina Wakisaka FMUSP-HC
dc.date.issued 2019
dc.identifier.citation ANAIS BRASILEIROS DE DERMATOLOGIA, v.94, n.2, p.133-146, 2019
dc.identifier.issn 0365-0596
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/32468
dc.description.abstract Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.
dc.language.iso eng
dc.publisher SOC BRASILEIRA DERMATOLOGIA
dc.relation.ispartof Anais Brasileiros de Dermatologia
dc.rights openAccess
dc.subject Antibodies; Autoimmunity; Hemidesmosomes; Pemphigoid; bullous; Skin diseases; vesiculobullous
dc.subject.other lichen-planus pemphigoides; intravenous immunoglobulin; topical corticosteroids; neurological diseases; ige autoantibodies; association; skin; childhood; mortality; expression
dc.title Bullous pemphigoid
dc.type article
dc.rights.holder Copyright SOC BRASILEIRA DERMATOLOGIA
dc.description.group LIM/53
dc.description.group LIM/56
dc.identifier.doi 10.1590/abd1806-4841.20199007
dc.identifier.pmid 31090818
dc.type.category original article
dc.type.version publishedVersion
hcfmusp.author MIYAMOTO, Denise:HC:ICHC
hcfmusp.author SANTI, Claudia Giuli:HC:ICHC
hcfmusp.author AOKI, Valeria:FM:MDT
hcfmusp.author MARUTA, Celina Wakisaka:FM:MDT
hcfmusp.origem.id WOS:000467378200002
hcfmusp.origem.id 2-s2.0-85066818108
hcfmusp.origem.id SCIELO:S0365-05962019000200133
hcfmusp.publisher.city RIO DE JANEIRO RJ
hcfmusp.publisher.country BRAZIL
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dc.description.index MEDLINE
dc.identifier.eissn 1806-4841
hcfmusp.citation.wos 0


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