CLAUDIA GIULI SANTI
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina
55 resultados
Resultados de Busca
Agora exibindo 1 - 10 de 55
- Analysis of the reactivity of indirect immunofluorescence in patients with pemphigus foliaceus and pemphigus vulgaris using rat bladder epithelium as a substrate(2011) ORTOLAN, Damaris G.; SOUZA, Danielle P. G.; AOKI, Valeria; SANTI, Claudia G.; GABBI, Tatiana V. B.; ICHIMURA, Ligia M. F.; MARUTA, Celina W.OBJECTIVES: To evaluate the reactivity of indirect immunofluorescence using rat bladder epithelium as a substrate in patients with pemphigus foliaceus and pemphigus vulgaris from the Department of Dermatology, University of Sao Paulo Medical School, Brazil. METHODS: Thirty-two patients (8 male and 24 female) from the Department of Dermatology, University of Sao Paulo Medical School, were selected. Three had mucosal pemphigus vulgaris, 20 had mucocutaneous pemphigus vulgaris, and 9 had pemphigus foliaceus. Patients' sera were tested by indirect immunofluorescence performed on human foreskin and rat bladder epithelium and by ELISA assays utilizing baculovirus-expressed recombinant desmoglein 3 and desmoglein 1. RESULTS: No patients with mucosal pemphigus vulgaris, 5 of 20 patients with mucocutaneous pemphigus vulgaris (25%) and 4 of 9 patients with pemphigus foliaceus (44%) had positive indirect immunofluorescence using rat bladder epithelium as a substrate. CONCLUSION: Indirect immunofluorescence using rat bladder epithelium as a substrate is recommended whenever a diagnosis of paraneoplastic pemphigus is considered. The identification of a subset of pemphigus foliaceus and pemphigus vulgaris patients that recognizes desmoplakins by this laboratory tool is critical to avoid the misdiagnosis of paraneoplastic pemphigus.
- Laryngeal involvement in pemphigus vulgaris: a proposed classification(2012) MAHMOUD, A.; MIZIARA, I. D.; COSTA, K. C.; SANTI, C. G.; MARUTA, C. W.; AOKI, V.Objective: (1) To investigate the incidence of laryngeal involvement in a large series of patients with pemphigus vulgaris, using endoscopic examination, (2) to describe the lesions, and (3) to establish a classification of laryngeal involvement in pemphigus vulgaris based on the location of the lesions. Study design: Prospective study. Methods: A total of 40 sequentially treated pemphigus vulgaris patients, diagnosed using clinical, histological and immunofluorescence criteria, were evaluated for laryngeal manifestations using endoscopic examination. The results were used to establish a graded classification of laryngeal involvement according to the location of the lesions. Results: Active laryngeal lesions (ulcers or blisters) were found in 16 patients (40 per cent). Of these, 37.5 per cent were classified as grade I, 20 per cent as grade II, 20 per cent as grade III and 17.5 per cent as grade IV. Conclusion: Laryngeal involvement is common in pemphigus vulgaris and must be considered at the point of diagnosis. Grade I lesions are the most frequent.
- 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.
- Drug reaction with eosinophilia and systemic symptoms/drug-induced hypersensitivity syndrome: clinical features of 27 patients(2015) AVANCINI, J.; MARAGNO, L.; SANTI, C. G.; CRIADO, P. R.BackgroundDrug reaction with eosinophilia and systemic symptoms (DRESS) [also called drug-induced hypersensitivity syndrome (DIHS)] includes severe reactions to drugs that need to be promptly recognized by physicians. AimTo explore heterogeneity in the clinical presentation of DRESS/DIHS at a large academic hospital in Latin America, using the criteria defined by the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scoring system. MethodsA retrospective medical record review of 60 patients with diagnostic suspicion of DRESS/DIHS admitted to our hospital between July 2008 and April 2012 was performed, including demographic data, clinical features, laboratory findings and treatment. ResultsOf the 60 patients, 27 fulfilled the criteria for DRESS/DIHS. Maculopapular exanthema (85.1%), fever (96.2%) and hepatic involvement (85.1%) were the most common features. Anticonvulsants were the most common causal drugs (77.7%); Phenytoin was the most common individual drug (44.4%), followed by carbamazepine (29.6%). All patients were treated initially with prednisone 1mg/kg/day. Mortality rate was 4%. ConclusionThe major findings of this study (to our knowledge the largest collection of data on DRESS/DIHS in Latin America) include a positive statistical association between presence of atypical lymphocytes and higher levels of alanine aminotransferase (P<0.001) and reinforce the importance of anticonvulsants in the pathogenesis of this severe reaction.
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 Giuli