RESUMO
INTRODUCTION: Mannose binding lectin (MBL) has been linked to predisposition to systemic lupus erythematosus (SLE) and to disease activity. Some studies found deposits of MBL in glomerular tissue of patients with lupus nephritis. There is no research about the deposition of MBL in skin. MATERIALS AND METHODS: Skin biopsies from lesional and non lesional skin of 4 discoid lupus erythematosus (DLE) and 10 SLE patients were submitted to immunofluorescence staining for IgG, IgA, IgM, C3, C4, C1q, C5b-9 and MBL. Charts were reviewed for demographic, clinical and serological data. Patients with SLE had disease activity measured by SLEDAI. RESULTS: MBL was found only in SLE lesional skin and its presence showed an association trend towards higher disease activity. Deposition of C5b-9 occurred in vessels only in patients with SLE (70%) and in the two patients with kidney involvement. CONCLUSIONS: MBL deposition was found in the lesional skin of SLE patients but not in SLE non lesional skin nor in DLE patients, and it seems to be less frequent and less strong than observed in the kidneys biopsies, suggesting that the complement participation in the pathophysiology of SLE process may not be the same in these two clinical manifestations.
Assuntos
Lúpus Eritematoso Discoide/patologia , Lúpus Eritematoso Sistêmico/patologia , Lectina de Ligação a Manose/genética , Pele/patologia , Adulto , Complemento C1q/genética , Complemento C1q/imunologia , Complemento C3/genética , Complemento C3/imunologia , Complemento C4/genética , Complemento C4/imunologia , Complexo de Ataque à Membrana do Sistema Complemento/genética , Complexo de Ataque à Membrana do Sistema Complemento/imunologia , Feminino , Expressão Gênica , Humanos , Imunoglobulinas/genética , Imunoglobulinas/imunologia , Lúpus Eritematoso Discoide/genética , Lúpus Eritematoso Discoide/imunologia , Lúpus Eritematoso Sistêmico/genética , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Lectina de Ligação a Manose/imunologia , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Pele/imunologiaRESUMO
A esclerodermia é uma doença autoimune com incidência de 0,4-2,7 para cada 100.000 pessoas com predominância pelo sexo feminino. Ela se apresenta de diversas formas, sendo a linear a forma mais prevalente em crianças, afetando 60% dos casos. Acomete membros e tronco, causando atrofia muscular e do subcutâneo, discrepância no comprimento dos membros, contraturas articulares, podendo levar inclusive à incapacidade física. Relatar uma indicação incomum para a gluteoplastia com resultado satisfatório usando- -se técnicas sedimentadas. J.B., feminino, 26 anos, branca, tratada de esclerodermia cutânea dos 3 aos 10 anos com remissão completa da atividade da doença. Apresentava sequela com atrofia cutânea, subcutânea e muscular somente no lado direito, localizada principalmente no sulco mamário, glúteo e face interna da coxa. A cirurgia foirealizada em dois tempos. O primeiro tempo consistiu em gluteoplastia unilateral de aumento com implante de silicone no plano intramuscular, lipoenxertia glútea e crural associados à mamoplastia de aumento bilateral. Num segundo momento uma nova lipoenxertia atenuou as assimetrias remanescentes. Devido à versatilidade e segurança da gluteoplastia associando-se inclusão de implante de silicone com a lipoenxertia é possível, em situações inusitadas, como esta paciente com uma sequela de esclerodermia unilateral, reestabelecer o contorno corporal com harmonia e resultados naturais
Scleroderma is an autoimmune disease with an incidence of 0.4 - 2.7 for every 100.000 people with preference by women. It shows itself in many ways, the linear form is more prevalent in children, affecting 60% of the cases. Affects limbs and trunk, causing muscle and subcutaneous atrophy, discrepancy in the length of the members, joint contractures, and may lead to physical disability. To report a rare indication for gluteoplasty with satisfactory results using known techniques. J. B., female, 26 years old, white, treated in cutaneous scleroderma from 3 to 10 years old with complete remission of the disease activity. She developed sequelae with cutaneous, subcutaneous and muscular atrophy only in the right side, located mainly in the inframammary fold, buttocks and inner thigh. The surgery was performed in two stages. The first time was a unilateral augmentation gluteoplasty with silicone gel implant positioned in the muscular plane, gluteal and crural lipofilling associated with bilateral augmentation mammoplasty. In a second moment a new lipofilling attenuated the remaining asymmetries. Due to the versatility and safety of gluteoplasty associating the inclusion of silicone gel implant with the lipofilling is possible, in unusual situations, such as this patient with a sequelae of unilateral scleroderma, reestablish the body contour with harmony and natural results