Assuntos
Penfigoide Bolhoso/complicações , Pênfigo/complicações , Administração Cutânea , Corticosteroides/administração & dosagem , Idoso de 80 Anos ou mais , Antifúngicos/administração & dosagem , Fármacos Dermatológicos/administração & dosagem , Quimioterapia Combinada , Humanos , Cetoconazol/administração & dosagem , Masculino , Penfigoide Bolhoso/tratamento farmacológico , Pênfigo/tratamento farmacológicoRESUMO
A 450-kDa human epidermal autoantigen was originally identified as a protein that reacted with the serum from an individual with a subepidermal blistering disease. Molecular cloning of this protein has now shown that it contains 5065 amino acids and has a molecular mass of 552 kDa. As reported previously this protein, which we call epiplakin, belongs to the plakin family, but it has some very unusual features. Epiplakin has 13 domains that are homologous to the B domain in the COOH-terminal region of desmoplakin. The last five of these B domains, together with their associated linker regions, are particularly strongly conserved. However, epiplakin lacks a coiled-coil rod domain and an amino-terminal domain, both of which are found in all other known members of the plakin family. Furthermore, no dimerization motif was found in the sequence. Thus, it is likely that epiplakin exists in vivo as a single-chain structure. Epitope mapping experiments showed that the original patient's serum recognized a sequence unique to epiplakin, which was not found in plectin. Immunofluorescence staining revealed the presence of epiplakin in whole sheets of epidermis and esophagus, in glandular cells of eccrine sweat and parotid glands and in mucous epithelial cells in the stomach and colon.
Assuntos
Autoantígenos/química , Autoantígenos/genética , Vesícula/genética , Proteínas do Citoesqueleto/química , Epiderme/imunologia , Sequência de Aminoácidos , Sequência de Bases , Vesícula/imunologia , Clonagem Molecular , Sequência Conservada , Desmoplaquinas , Feminino , Biblioteca Gênica , Células HeLa , Humanos , Masculino , Dados de Sequência Molecular , Peso Molecular , Fases de Leitura Aberta , Especificidade de Órgãos , Homologia de Sequência de AminoácidosRESUMO
A 40-year-old man developed squamous cell carcinoma on a perianal lesion of linear porokeratosis after renal transplantation. The tumor metastasized to the left inguinal lymph node 25 months after the primary tumor was excised. p53 overexpression was observed in the tumor cells, but not in the porokeratotic lesion. Interestingly, continuous subcutaneous infusion of peplomycin for the lymph node metastasis significantly improved the warty lesions of porokeratosis. In this patient, immunosuppressive agents might have accelerated the development of carcinoma on a skin area with malignant potential.