CLAUDIA GIULI SANTI
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina
37 resultados
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Agora exibindo 1 - 10 de 37
- Bullous pemphigoid(2019) MIYAMOTO, Denise; SANTI, Claudia Giuli; AOKI, Valeria; MARUTA, Celina WakisakaBullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.
bookPart Dermatoses potencialmente graves na emergência(2017) VASCONCELOS, Roberta; CRIADO, Paulo Ricardo; SANTI, Claudia Giuli- Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)/Drug-Induced Hypersensitivity Syndrome (DIHS): a review of current concepts(2012) CRIADO, Paulo Ricardo; CRIADO, Roberta Fachini Jardim; AVANCINI, Joao de Magalhaes; SANTI, Claudia GiuliThe Drug Reaction with Eosinophilia and Systemic Symptoms syndrome, also known as Drug Induced Hypersensitivity Syndrome presents clinically as an extensive mucocutaneous rash, accompanied by fever, lymphadenopathy, hepatitis, hematologic abnormalities with eosinophilia and atypical lymphocytes, and may involve other organs with eosinophilic infiltration, causing damage to several systems, especially to the kidneys, heart, lungs, and pancreas. Recognition of this syndrome is of paramount importance, since the mortality rate is about 10% to 20%, and a specific therapy may be necessary. The pathogenesis is related to specific drugs, especially the aromatic anticonvulsants, altered immune response, sequential reactivation of herpes virus and association with HLA alleles. Early recognition of the syndrome and withdrawal of the offending drug are the most important and essential steps in the treatment of affected patients. Corticosteroids are the basis of the treatment of the syndrome, which may be associated with intravenous immunoglobulin and, in selected cases, Ganciclovir. The article reviews the current concepts involving this important manifestation of adverse drug reaction.
- Trichosporon inkin as an Emergent Pathogen in Patients With Severe Pemphigus(2015) ALMEIDA JUNIOR, Joao Nobrega de; OLIVEIRA, Renata Buccheri de; DUARTE, Amaro; MOTTA, Adriana Lopes; ROSSI, Flavia; FIGUEIREDO, Dulce Sachiko Yamamoto de; NEGRO, Gilda Maria Barbaro Del; AOKI, Valeria; MARUTA, Celina Wakisaka; SANTI, Claudia Giuli; BENARD, GilIMPORTANCE To our knowledge, these are the first reports of bloodstream infections by Trichosporon inkin in patients with pemphigus. OBSERVATIONS Trichosporon inkin, a novel organism causing bloodstream infection, was detected in 2 patients with pemphigus. An elderly man with pemphigus foliaceus died despite treatment with liposomal amphotericin B, 3mg/kg/d, and a young girl with pemphigus vulgaris responded to treatment with voriconazole, 8mg/kg/d, for 24 days. One of the T inkin isolates had a minimal inhibitory concentration of 2mg/L against amphotericin B, suggesting resistance to the drug. CONCLUSIONS AND RELEVANCE Delayed suspicion of invasive infection by T inkin may result in a poor outcome in patients with severe forms of pemphigus. This opportunistic infection is highly refractory to conventional potent antifungal treatment.
bookPart Reações cutâneas medicamentosas(2013) VASCONCELOS, Roberta; CRIADO, Paulo Ricardo; SANTI, Claudia GiulibookPart Afecções das mucosas(2019) WU, Isabelle I. Hue; FERREIRA, Paula Silva; SANTI, Claudia Giuli; NICO, Marcello Menta SimonsenbookPart Erupções paravirais(2022) YENDO, Tatiana Mina; SANTI, Claudia GiulibookPart Reações Medicamentosas(2016) SANTI, Claudia Giuli; DIAS, Natasha Favoretto; AVANCINI, JoãobookPart Reações cutâneas medicamentosas(2017) VASCONCELOS, Roberta; CRIADO, Paulo Ricardo; SANTI, Claudia GiulibookPart Reações Cutâneas Medicamentosas(2016) VASCONCELOS, Roberta; CRIADO, Paulo Ricardo; SANTI, Claudia Giuli