CLAUDIA GIULI SANTI
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina
5 resultados
Resultados de Busca
Agora exibindo 1 - 5 de 5
bookPart Erupções paravirais(2022) YENDO, Tatiana Mina; SANTI, Claudia Giuli- Epidermolysis bullosa acquisita(2022) MIYAMOTO, Denise; GORDILHO, Juliana Olivieri; SANTI, Claudia Giuli; PORRO, Adriana MariaEpidermolysis bullosa acquisita is a rare autoimmune disease, characterized by the synthesis of anti-collagen VII autoantibodies, the main component of hemidesmosome anchoring fibrils. The antigen-antibody binding elicits a complex inflammatory response, which culminates in the loss of dermo-epidermal adhesion of the skin and/or mucous membranes. Skin fragility with bullae, erosions, and milia in areas of trauma characterizes the mechanobullous form of the disease. In the inflammatory form of epidermolysis bullosa acquisita, urticarial inflammatory plaques with tense bullae, similar to bullous pemphigoid, or mucosal lesions can determine permanent scars and loss of functionality in the ocular, oral, esophageal, and urogenital regions. Due to the similarity of the clinical findings of epidermolysis bullosa acquisita with other diseases of the pemphigoid group and with porphyria cutanea tarda, the diagnosis is currently confirmed mainly based on the clinical correlation with histopathological findings (pauci-inflammatory subepidermal cleavage or with a neutrophilic infiltrate) and the demonstration of the presence of anti-collagen VII IgG in situ by direct immunofluorescence, or circulating anti-collagen VII IgG through indirect immunofluorescence and/or ELISA. There is no specific therapy for epidermolysis bullosa acquisita and the response to treatment is variable, usually with complete remission in children and a worse prognosis in adults with mucosal involvement. Systemic corticosteroids and immunomodulators (colchicine and dapsone) are alternatives for the treatment of mild forms of the disease, while severe forms require the use of corticosteroid therapy associated with immunosuppressants, intravenous immunoglobulin, and rituximab. (C) 2022 Sociedade Brasileira de Dermatologia.
- Considerations on Immunization and Immunosuppression of Patients With Autoimmune Blistering Diseases During COVID-19 Pandemic in Brazil: Case Report(2022) MIYAMOTO, Denise; SANTI, Claudia Giuli; MARUTA, Celina Wakisaka; AOKI, ValeriaAutoimmune blistering diseases comprise a rare group of potentially life-threatening dermatoses. Management of autoimmune disorders poses a challenge in terms of achieving disease control and preventing adverse events. Treatment often requires an individualized approach considering disease severity, age, comorbidities, and infectious risk especially in the context of the ongoing COVID-19 pandemic. Knowledge regarding SARS-CoV-2 infection is still evolving and no specific antiviral therapy is available yet. We report four patients with active disease that required adjustment of treatment during the pandemic to discuss the use of immunosuppressants and immunobiologics, weighing potential risks and benefits of each therapy modality and vaccination status.
- Systemic retinoid treatment in a case of hyperkeratotic lichenoid paraneoplastic pemphigus(2022) YENDO, Tatiana Mina; MIYAMOTO, Denise; SANTI, Claudia G.
bookPart Farmacodermias(2022) ALVES, João de Magalhães Avancini Ferreira; SANTI, Claudia Giuli