Clinical and immunological profile of umbilical involvement in pemphigus vulgaris and pemphigus foliaceus

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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 OLIVEIRA JUNIOR, J. V. FMUSP-HC
MARUTA, C. W. FMUSP-HC
SOUSA JR., J. X. FMUSP-HC
SANTI, C. G. FMUSP-HC
VALENTE, N. Y. S. FMUSP-HC
ICHIMURA, L. M. F. FMUSP-HC
PERIGO, A. M. FMUSP-HC
AOKI, V. FMUSP-HC
dc.date.issued 2013
dc.identifier.citation CLINICAL AND EXPERIMENTAL DERMATOLOGY, v.38, n.1, p.20-24, 2013
dc.identifier.issn 0307-6938
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/1917
dc.description.abstract Background. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune vesicobullous disorders with IgG autoantibodies directed against desmoglein (Dsg)1 and 3, which lead to intraepidermal acantholysis. Aim. To characterize the clinical and immunological profile of patients with PF or PV with umbilical involvement. Methods. In total, 10 patients (7 women, 3 men; age range 2470 years, disease duration 316 years) diagnosed with either PV (n = 5) or mucocutaneous PF (n = 5) were assessed according to their clinical features, histopathology and immunological findings [direct and indirect immunofluorescence (DIF and IIF) and ELISA with recombinant Dsg1 and Dsg3]. Results. Erythema, erosions, crusts and vegetating skin lesions were the main clinical features of the umbilical region. DIF of the umbilical region gave positive results for intercellular epidermal IgG and C3 deposits in eight patients and for IgG alone in the other two. Indirect immunofluorescence with IgG conjugate showing the typical pemphigus pattern was positive in all 10 patients, with titres varying from 1 : 160 to 1 : 2560. ELISA with recombinant Dsg1 gave scores of 24266 in PF and 0270 in PV. Reactivity to recombinant Dsg3 was positive in all five patients with PV (ELISA 2298) and was negative in all PF sera. Conclusions. All 10 patients with pemphigus with umbilical presentation had the clinical and immunopathological features of either PF or PV. This peculiar presentation, not yet completely elucidated, has rarely been reported in the literature. A possible explanation for this unique presentation may be the presence of either novel epitopes or an association with embryonic or scar tissue located in the umbilical-cord region.
dc.description.sponsorship · CNPq [476954/2010-0]
dc.language.iso eng
dc.publisher WILEY-BLACKWELL
dc.relation.ispartof Clinical and Experimental Dermatology
dc.rights restrictedAccess
dc.subject.other hla-g; cell-adhesion; desmoglein-4; trophoblast; phenotype; cadherin; vegetans; antigen; genes
dc.title Clinical and immunological profile of umbilical involvement in pemphigus vulgaris and pemphigus foliaceus
dc.type article
dc.rights.holder Copyright WILEY-BLACKWELL
dc.description.group LIM/53
dc.description.group LIM/56
dc.identifier.doi 10.1111/j.1365-2230.2012.04468.x
dc.identifier.pmid 23083306
dc.type.category original article
dc.type.version publishedVersion
hcfmusp.author OLIVEIRA JUNIOR, J. V.:FM:
hcfmusp.author MARUTA, C. W.:FM:MDT
hcfmusp.author SOUSA JR., J. X.:FM:
hcfmusp.author SANTI, C. G.:HC:ICHC
hcfmusp.author VALENTE, N. Y. S.:HC:LIM/53
hcfmusp.author ICHIMURA, L. M. F.:HC:ICHC
hcfmusp.author PERIGO, A. M.:HC:ICHC
hcfmusp.author AOKI, V.:FM:MDT
hcfmusp.origem.id 2-s2.0-84872865604
hcfmusp.origem.id WOS:000312543400004
hcfmusp.publisher.city HOBOKEN
hcfmusp.publisher.country USA
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dc.description.index MEDLINE
hcfmusp.citation.scopus 3
hcfmusp.citation.wos 3


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