RESUMO
We describe a case of fatal systemic amyloidosis presenting with mucocutaneous bullous lesions in a patient with IgA kappa monoclonal gammopathy. The amyloid plaques were composed of an unusual mixture of immunoglobulin kappa light chain and amyloid A proteins. Whereas oesophageal and oropharyngeal blisters are known to occur in several types of bullous dermatoses, to our knowledge this is the first report of oesophagopharyngeal blisters complicating bullous amyloidosis.
Assuntos
Amiloidose/metabolismo , Dermatopatias Vesiculobolhosas/metabolismo , Idoso de 80 Anos ou mais , Amiloidose/complicações , Amiloidose/patologia , Vesícula/etiologia , Doenças do Esôfago/etiologia , Evolução Fatal , Feminino , Humanos , Cadeias Leves de Imunoglobulina/análise , Cadeias kappa de Imunoglobulina/análise , Paraproteinemias/complicações , Doenças Faríngeas/etiologia , Proteína Amiloide A Sérica/análise , Dermatopatias Vesiculobolhosas/complicações , Dermatopatias Vesiculobolhosas/patologiaAssuntos
Doenças Autoimunes/terapia , Doença Enxerto-Hospedeiro/complicações , Tacrolimo/análogos & derivados , Absorção , Administração Tópica , Doenças Autoimunes/complicações , Transplante de Medula Óssea/efeitos adversos , Pré-Escolar , Doença Enxerto-Hospedeiro/tratamento farmacológico , Humanos , Masculino , Dermatopatias/complicações , Dermatopatias/tratamento farmacológico , Tacrolimo/farmacocinética , Tacrolimo/uso terapêutico , Tacrolimo/toxicidadeAssuntos
Doença Enxerto-Hospedeiro/patologia , Hipopigmentação/etiologia , Adolescente , Transplante de Medula Óssea/efeitos adversos , Transplante de Medula Óssea/imunologia , Humanos , Hipotireoidismo , Masculino , Fatores de Tempo , Condicionamento Pré-Transplante/efeitos adversos , Condicionamento Pré-Transplante/métodosRESUMO
A retrospective analysis of 25 infants and young children with anogenital warts was performed by chart review and telephone interview. Fifteen of 17 patients treated with podofilox 0.5% gel and 6 of 8 patients treated with imiquimod 5% cream improved or cleared with therapy. Only one patient stopped treatment because of irritation. Our experience suggests that these agents can be used safely and effectively in young children. Controlled prospective studies should be undertaken to further evaluate the use of podofilox and imiquimod in the treatment of symptomatic anogenital warts in children.
Assuntos
Adjuvantes Imunológicos/uso terapêutico , Aminoquinolinas/uso terapêutico , Antineoplásicos Fitogênicos/uso terapêutico , Condiloma Acuminado/tratamento farmacológico , Podofilotoxina/uso terapêutico , Adjuvantes Imunológicos/administração & dosagem , Administração Tópica , Aminoquinolinas/administração & dosagem , Antineoplásicos Fitogênicos/administração & dosagem , Pré-Escolar , Feminino , Géis , Humanos , Imiquimode , Lactente , Masculino , Pomadas , Podofilotoxina/administração & dosagem , Estudos Retrospectivos , Resultado do TratamentoAssuntos
Carcinoma Basocelular/genética , Histiocitoma Fibroso Benigno/genética , Proteínas de Membrana/genética , Neoplasias Cutâneas/genética , Genes Supressores de Tumor , Humanos , Perda de Heterozigosidade , Proteínas de Neoplasias/genética , Receptores Patched , Receptor Patched-1 , Receptores de Superfície CelularRESUMO
Using immunohistochemical techniques, we mapped and quantified the distribution of Langerhans cells (LCs) within the follicular epithelium of normal human skin in serial horizontal sections. Ten skin biopsies from disparate, disease-free sites from individuals of various skin types were stained with antibody to CD1a. LCs concentrated in the infundibular epithelium (x=16.16 cells), including the follicular bulge, and extended into the germinative sebaceous epithelium (x=8.84). In contrast, rare LCs (x=1.06) were observed in the follicular epithelium below the entry of sebaceous glands into the follicle. LCs were absent in bulbar epithelium. This infundibulocentric distribution of LCs corresponds to the pattern of follicular inflammation in the scarring folliculitides of lupus erythematosus and lichen planopilaris, as well as allogeneic graft versus host reaction and infundibulofolliculitis of atopy. Follicular LCs may act as the trigger and/or target for these T cell-mediated inflammatory processes.
Assuntos
Folículo Piloso/citologia , Células de Langerhans/citologia , Anticorpos Monoclonais , Antígenos CD1/análise , Células Epidérmicas , Epiderme/imunologia , Células Epiteliais/imunologia , Feminino , Folículo Piloso/imunologia , Humanos , Técnicas Imunoenzimáticas , Células de Langerhans/imunologia , Masculino , Glândulas Sebáceas/citologia , Glândulas Sebáceas/imunologiaRESUMO
Although it appears that survival has been increased and hospital stays have been decreased in elderly patients with burn injuries, limited information is available on the functional status of these patients at the time of discharge from the hospital. Because this information is necessary to assess more fully the success or failure of current modes of therapy, we have reviewed the records of 99 surviving patients with burn injuries over the age of 55 years. These patients had a mean age 71.8 years and a mean +/- SD burn size of 8.9% +/- 8.6%, with 36 patients having burns over greater than 10% of body surface area and 14 patients having burns over greater than 20%. The mean hospital stay of these patients was 16.9 days, and 75% of the patients required surgery to heal the burn wounds. Since just five (5%) of the 99 patients required nursing home placement at discharge, it appears that the majority of elderly patients with burn injuries recover sufficiently from the injuries to resume their preinjury life-style.