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BACKGROUND: Generalized pustular psoriasis (GPP) is a rare and severe phenotype of psoriasis characterized by sudden outbreak of widespread coalescent sterile pustules associated with a spectrum of systemic symptoms. OBJECTIVE: We aimed to describe the epidemiology and treatment of GPP in Brazil from the public health care system perspective. METHODS: This was a retrospective public claims database study, using outpatient and inpatient databases, with information from January 2018 to August 2020, based on records of health resource utilization by patients with GPP. Outpatient treatment regimens and fatal inpatient outcomes were described. RESULTS: In total, 1458 outpatients of all ages were identified, of whom 53% were women. We estimated the GPP prevalence in Brazil to be between 0.7 and 0.9 per 100,000. Acitretin was the most commonly dispensed drug. Of all the outpatients, 769 outpatients could be tracked in the inpatient database, and 151 had hospital admissions during the study period. Of them, 5.3% had a fatal outcome during hospitalization. A primary skin condition or an infection was the most frequent hospitalization cause. LIMITATION: The International Classification of Diseases codes for GPP and psoriasis have not been previously validated in this context. CONCLUSION: GPP is a rare disease in Brazil and affects individuals of all ages and both sexes. Hospitalizations and disease-related deaths highlight the need for its prompt diagnosis, close medical follow-up, and effective treatment.
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INTRODUÇÃO: O Sarcoma de Kaposi (SK) é a neoplasia mais frequente dos doentes com Aids. É causada pelo herpes-vírus 8 (HHV-8). As células dendríticas plasmocitóides (CDp) são especializadas na produção de interferon tipo 1 e participam da resposta imune aos vírus. Os receptores "toll-like" são os principais receptores de reconhecimento de padrão, sendo que os receptores toll-like (TLR) 3 e 9 têm função no reconhecimento de vírus. O D2-40 é o anticorpo que reconhece a podoplanina, uma proteína transmembrana, presente no endotélio linfático e que tem função na imunidade. OBJETIVO: Demonstrar e comparar os componentes da imunidade inata: CDp e TLR 3 e 9, nas lesões cutâneas de SK associado a Aids e esporádico. Identificar a presença do HHV-8 nas CDp. Verificar o componente endotelial linfático na progressão das lesões de SK e comparar a expressão dos elementos da imunidade inata estudados, nas lesões com menor e maior componente endotelial linfático. MÉTODOS: Estudo retrospectivo de 50 biopsias de pacientes com diagnóstico de SK, todos com comprovação pelo exame histopatológico e demonstração do antígeno nuclear associado à latência (LANA) do HHV-8. Foram avaliados 11 biopsias de SK da forma clássica (SKc), 22 lesões de doentes com Aids (SK-Aids) e de 17 de doentes com Aids submetidos a tratamento com terapia antirretroviral altamente eficaz (SK-Aids/HAART). Os espécimes foram submetidos a exame por técnica imuno-histoquímica para evidenciar a presença de CDp (anticorpo CD303/BDCA-2), a expressão de TLR 3 e 9, bem como de podoplanina (anticorpo D2-40). Foi realizada também técnica de dupla marcação com CD303 e LANA, objetivando a identificação de CDp infectadas pelo HHV-8.Vinte e três espécimes de granuloma piogênico constituíram o grupo controle. A população de CDp e expressão de TLR 3 e TLR 9 também foi comparada nas lesões cutâneas de SK de doentes com e sem comprometimento visceral pela neoplasia; lesões não tumorais (máculo-papulares/placas)...
Introduction: Kaposi's sarcoma (KS) is the most common Aids-associated malignancy. It is caused by human herpesvirus-8. Plasmacytoid dendritic cells (pDC) are professional interferon producing cells, and participate in the immune response against viruses. Toll-like receptors (TLR) are the main pattern recognition receptors, and TLR 3 and TLR 9 participate in the recognition of viruses. Podoplanin, recognized by antibody D2-40, is a transmembrane protein identified on lymphatic endothelial cells with functions inimmunity. Objective: Demonstrate and compare some innate immunity components: pDC, TLR 3 and TLR 9, in cutaneous lesions of Aids-associated Kaposi's sarcoma and classic Kaposi's sarcoma. Identify the infection of pDC by HHV-8. Compare the lymphatic endothelial component in the course of tumor progression and compare the expression of innate immunity elements in lesions with a predominance of lymphatic endothelial components or not. Methods: Retrospective study of 50 biopsies diagnosed as Kaposi's sarcoma withpositive staining for latency-associated nuclear antigen (LANA) of HHV-8. Eleven classic KS, 22 Aids-associated KS and 17 Aids-associated KS from patients undergoing highly active antiretroviral therapy (HAART) were assessed. Paraffinembedded tissue was submitted to immunohistochemistry technique in order to demonstrate pDC (CD303/BDCA-2 antibody), expression of TLR 3, TLR 9 and podoplanin (D2-40 antibody). We performed double staining with CD303 and LANA in order to identify pDC infection with HHV-8. Twenty-three pyogenic granuloma(PG) specimens were analyzed as a control group. Plasmacytoid dendritic cells population, TLR 3 and TLR 9 expressions were compared between patients with and without visceral disease, nodular stageandpatch/plaque stage and according to bloodlymphocytes T CD4 count(< and >=350 cells/mm3)...
