Clinical and immunological profile of umbilical involvement in pemphigus vulgaris and pemphigus foliaceus
Carregando...
Citações na Scopus
5
Tipo de produção
article
Data de publicação
2013
Título da Revista
ISSN da Revista
Título do Volume
Editora
WILEY-BLACKWELL
Citação
CLINICAL AND EXPERIMENTAL DERMATOLOGY, v.38, n.1, p.20-24, 2013
Resumo
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.
Palavras-chave
Referências
- AMAGAI M, 1991, CELL, V67, P869, DOI 10.1016/0092-8674(91)90360-B
- ARNEMANN J, 1991, GENOMICS, V10, P640, DOI 10.1016/0888-7543(91)90446-L
- BUXTON RS, 1993, J CELL BIOL, V121, P481, DOI 10.1083/jcb.121.3.481
- Cowley CME, 1997, GENOMICS, V42, P208, DOI 10.1006/geno.1997.4718
- Culton DA, 2008, J AUTOIMMUN, V31, P311, DOI 10.1016/j.jaut.2008.08.003
- Ding X, 1997, J INVEST DERMATOL, V109, P592, DOI 10.1111/1523-1747.ep12337524
- ELLIS SA, 1986, IMMUNOLOGY, V59, P595
- Hunt JS, 2005, FASEB J, V19, P681, DOI 10.1096/fj.04-2078rev
- Hunt JS, 2006, IMMUNOL REV, V213, P36, DOI 10.1111/j.1600-065X.2006.00436.x
- JONES JCR, 1986, P NATL ACAD SCI USA, V83, P7282, DOI 10.1073/pnas.83.19.7282
- Kljuic A, 2003, CELL, V113, P249, DOI 10.1016/S0092-8674(03)00273-3
- KOVATS S, 1990, SCIENCE, V248, P220, DOI 10.1126/science.2326636
- LEROY D, 1982, ANN DERMATOL VENER, V109, P549
- Morales PJ, 2003, J IMMUNOL, V171, P6215
- Petroff MG, 2001, J REPROD IMMUNOL, V56, P3
- Pistoia V, 2007, SEMIN CANCER BIOL, V17, P469, DOI 10.1016/j.semcancer.2007.07.004
- Ucley MC, 1999, JAMA-J AM MED ASSOC, V282, P572
- Whittock NV, 2003, J INVEST DERMATOL, V120, P523, DOI 10.1046/j.1523-1747.2003.12113.x
- WINKELMANN RK, 1979, ARCH DERMATOL, V115, P446, DOI 10.1001/archderm.115.4.446