Autoimmunity to type VII collagen in SKH1 mice is independent of regulatory T cells

SUMMARY: Epidermolysis bullosa acquisita is an autoimmune blistering disease characterized by circulating and skin basement membrane-bound IgG autoantibodies to type VII collagen, a major structural protein of the dermal–epidermal junction. Regulatory T cells (T(reg)) suppress self antigen-mediated autoimmune responses. To investigate the role of T(reg) in the the autoimmune response to type VII collagen in a mouse model, a monoclonal antibody against mouse CD25 was used to deplete T(reg). A recombinant mouse type VII collagen NC1 domain protein and mouse albumin were used as antigens. SKH1 mice were used as a testing host. Group 1 mice received NC1 immunization and were functionally depleted of T(reg); group 2 mice received NC1 immunization and rat isotype control; and group 3 mice received albumin immunization and were functionally depleted of T(reg). Results demonstrated that anti-NC1 IgG autoantibodies with high titres, as determined by enzyme-linked immunosorbent assay and Western blotting, developed in all mice immunized with NC1 (groups 1 and 2), but were undetected in group 3 mice. The predominant subclasses of anti-NC1 autoantibodies were IgG1, IgG2a and IgG2b; furthermore, these antibodies carried only the kappa light chain. IgG autoantibodies in the sera of NC1-immunized mice reacted with mouse skin basement membrane in vitro and deposited in skin basement membrane in vivo as detected by indirect and direct immunofluorescence microscopy, respectively. Our data suggest that the development of autoimmunity against type VII collagen in mice is independent of T(reg) function and the autoimmune response is mediated by both Th1 and Th2 cells. We speculate that the basement membrane deposition of IgG may eventually lead to blister development

Tratamento da doença de Hailey-Hailey com toxina botulínica tipo A Hailey-Hailey disease treatment with Botulinum toxin type A

Duas irmãs com doença de Hailey-Hailey, com lesões recorrentes - uma em axilas e outra em região inguinal -, e resposta limitada aos tratamentos clássicos. Elas foram tratadas com aplicação de toxina botulínica tipo A. Observamos que houve importante melhora na paciente tratada na região inguinal e remissão completa na paciente em cujas axilas sofreram tratamento. Além disso, foi possível poupar uso de antibióticos sistêmicos e corticoides tópicos. O alto custo é um fator restritivo para uso rotineiro e estudos maiores são necessários para definir eficácia e relação custo-benefício dessa intervenção.<br>Two sisters with recurrent lesions, one on axillae and other on the groin, and with limited response to classical treatments were treated with injections botulinum toxin type A. We observed marked improvement in the patient treated in the groin and complete remission in the patient treated in the axillae. It was possible to spare the use of systemic antibiotics and topical corticosteroids. The high cost is a restrictive factor to routine use and large studies are necessary to access efficacy and cost benefit profile

Consumer ethnocentrism: Reconceptualization and cross-cultural validation

Consumer ethnocentrism (CE) is a popular construct in international marketing research and is generally measured using the CETSCALE, a reliable scale with proven predictive validity but with limited evidence about its construct validity, dimensionality and cross-cultural measurement invariance. This note addresses these gaps by reconceptualizing CE as an attitude construct consisting of three dimensions: (1) affective reaction, (2) cognitive bias and (3) behavioral preference. A revised CE scale (CES) is developed and tested using two empirical studies with adult consumers from four different countries (China, India, UK and USA), showing that CES is a reliable, valid and cross-culturally invariant scale and it explains greater variance than the CETSCALE and other similar scales, in customer evaluations and behavioral intentions for a wide range of products and services