Clinical and immunopathological evaluation of epidermolysis bullosa acquisita

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorDELGADO, L.
dc.contributor.authorAOKI, V.
dc.contributor.authorSANTI, C.
dc.contributor.authorGABBI, T.
dc.contributor.authorSOTTO, M.
dc.contributor.authorMARUTA, C.
dc.date.accessioned2017-11-27T16:26:18Z
dc.date.available2017-11-27T16:26:18Z
dc.date.issued2011
dc.description.abstractP>Background. Epidermolysis bullosa acquisita (EBA) is a subepidermal blistering disease with IgG antibodies against collagen VII. The disease is heterogeneous and can lead to significant morbidity. Aim. To characterize the clinical and laboratory profile of patients with EBA from Sao Paulo, Brazil. Methods. In total, 12 patients (mean age 24 years) were analysed for cutaneous and mucosal involvement, laboratory data and response to treatment. Results. Mucosal involvement occurred in 11 of the 12 patients (eyes in 4/12, nose in 4/9, pharynx-larynx in 5/9 and oesophagus in 4/10; 3 patients did not undergo nasopharyngeal examination and 2 paediatric patients did not undergo endoscopy). Using direct immunofluorescence, different patterns of deposits were found at the basement membrane zone: IgG (12/12), IgA (6/12), IgM (4/12), C3 (11/12). Indirect immunofluorescence (IIF) was positive in 6 of 12 patients, and IIF on salt-split skin detected dermal deposition in 10 of 12 patients. Antinuclear antibodies were found in 3 of 12 patients, but none of them fulfilled the criteria for systemic lupus erythematosus. After treatment, total remission was achieved in three patients and partial remission in five (three were maintained on minimal treatment, one on the full treatment and one was able to come off treatment). Two patients were lost to follow-up and the remaining two had disease flares. Complications were mainly mucosal (oesophageal stenosis, laryngeal synechia, symblephara and trichiasis). Conclusions. Mucosal involvement in EBA is a determining factor for disease morbidity. Complete evaluation of the patient, focusing on both cutaneous and extracutaneous sites is essential, as EBA may evolve to refractory disease, severely compromising its outcome.
dc.description.indexMEDLINE
dc.identifier.citationCLINICAL AND EXPERIMENTAL DERMATOLOGY, v.36, n.1, p.12-18, 2011
dc.identifier.doi10.1111/j.1365-2230.2010.03845.x
dc.identifier.issn0307-6938
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/22943
dc.language.isoeng
dc.publisherWILEY-BLACKWELL PUBLISHING, INC
dc.relation.ispartofClinical and Experimental Dermatology
dc.rightsrestrictedAccess
dc.rights.holderCopyright WILEY-BLACKWELL PUBLISHING, INC
dc.subject.othersystemic-lupus-erythematosus
dc.subject.othervii collagen
dc.subject.otherchildhood
dc.subject.otherantigen
dc.subject.otherautoantibodies
dc.subject.otherend
dc.subject.wosDermatology
dc.titleClinical and immunopathological evaluation of epidermolysis bullosa acquisita
dc.typearticle
dc.type.categoryoriginal article
dc.type.versionpublishedVersion
dspace.entity.typePublication
hcfmusp.author.externalDELGADO, L.:Univ Sao Paulo, Hosp Clin Sao Paulo, Dept Dermatol, BR-05403900 Sao Paulo, Brazil
hcfmusp.citation.scopus35
hcfmusp.contributor.author-fmusphcVALERIA AOKI
hcfmusp.contributor.author-fmusphcCLAUDIA GIULI SANTI
hcfmusp.contributor.author-fmusphcTATIANA VILLAS BOAS GABBI
hcfmusp.contributor.author-fmusphcMIRIAN NACAGAMI SOTTO
hcfmusp.contributor.author-fmusphcCELINA WAKISAKA MARUTA
hcfmusp.description.beginpage12
hcfmusp.description.endpage18
hcfmusp.description.issue1
hcfmusp.description.volume36
hcfmusp.origemWOS
hcfmusp.origem.pubmed20456382
hcfmusp.origem.scopus2-s2.0-78650114213
hcfmusp.origem.wosWOS:000285154200003
hcfmusp.publisher.cityMALDEN
hcfmusp.publisher.countryUSA
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