Generalized pustular psoriasis (von Zumbusch)

Abstract Generalized pustular psoriasis (von Zumbusch) is a rare and acute eruption characterized by multiple sterile pustules over an erythematous and edematous background, eventually associated with psoriasis vulgaris. Classically, it manifests as a potentially severe systemic picture and demands prompt diagnosis and intervention. The duration of each flare-up and intervals between the pustular episodes is extremely variable. Recently, genetic abnormalities have been identified mainly in the familial and early variants of this disease. The therapeutic arsenal is limited; however, new drugs being evaluated aim to control both pustular flare-ups and disease recurrences.

Brazilian recommendations on the safety and effectiveness of the yellow fever vaccination in patients with chronic immune-mediated inflammatory diseases

Abstract Background: In Brazil, we are facing an alarming epidemic scenario of Yellow fever (YF), which is reaching the most populous areas of the country in unvaccinated people. Vaccination is the only effective tool to prevent YF. In special situations, such as patients with chronic immune-mediated inflammatory diseases (CIMID), undergoing immunosuppressive therapy, as a higher risk of severe adverse events may occur, assessment of the risk-benefit ratio of the yellow fever vaccine (YFV) should be performed on an individual level. Main body of the abstract: Faced with the scarcity of specific orientation on YFV for this special group of patients, the Brazilian Rheumatology Society (BRS) endorsed a project aiming the development of individualized YFV recommendations for patients with CIMID, guided by questions addressed by both medical professionals and patients, followed an internationally validated methodology (GIN-McMaster Guideline Development). Firstly, a systematic review was carried out and an expert panel formed to take part of the decision process, comprising BRS clinical practitioners, as well as individuals from the Brazilian Dermatology Society (BDS), Brazilian Inflammatory Bowel Diseases Study Group (GEDIIB), and specialists on infectious diseases and vaccination (from Tropical Medicine, Infectious Diseases and Immunizations National Societies); in addition, two representatives of patient groups were included as members of the panel. When the quality of the evidence was low or there was a lack of evidence to determine the recommendations, the decisions were based on the expert opinion panel and a Delphi approach was performed. A recommendation was accepted upon achieving ≥80% agreement among the panel, including the patient representatives. As a result, eight recommendations were developed regarding the safety of YFV in patients with CIMID, considering the immunosuppression degree conferred by the treatment used. It was not possible to establish recommendations on the effectiveness of YFV in these patients as there is no consistent evidence to support these recommendations. Conclusion: This paper approaches a real need, assessed by clinicians and patient care groups, to address specific questions on the management of YFV in patients with CIMID living or traveling to YF endemic areas, involving specialists from many areas together with patients, and might have global applicability, contributing to and supporting vaccination practices. We recommended a shared decision-making approach on taking or not the YFV.

Limbal transplantation at a tertiary hospital in Brazil: a retrospective study / Transplante de limbo em hospital terciário no Brasil: estudo retrospectivo

ABSTRACTPurpose:Epidemiological analysis of limbal transplantation surgeries performed in the Ophthalmologic Hospital of Sorocaba.Methods:Retrospective medical records review of 30 patients who underwent limbal stem cell transplants between January 2003 and March 2008. Cases involving conjunctival limbal autograft were classified as group I, and those involving conjunctival limbal allograft as group II.Results:Two patients were excluded due to incomplete data during postoperative follow-up. Of the total sample of 28 patients, 53.6% constituted group I, whereas 46.4% were included in group II. Males were predominant (67.9%), and right eyes were the most prevalent (67.9%). The mean age was 40.3 years. Unilateral cases accounted for 60.7%. The most frequent pathology causing limbal system failure was chemical burns (53%). The mean length of time from diagnosis to surgery was 11.18 years. The limbal graft and amniotic membrane were associated in 75% of all cases, and tarsorrhaphy in 57.1%. The average follow-up period was 24.84 months. The uncorrected visual acuity improved in 38% of the cases, was unchanged in 28.5%, and deteriorated in 33.3%. There was no persistent epithelial defect in 75% of the patients. The conjunctivalization rate was similar between the groups (53.3% and 58.3%, respectively). The transparency improved in only 38.4% of the cases, and 28.5% of the surgeries performed were successful. The most prevalent complication was persistent epithelial defect, which occurred in 25% of the patients, followed by corneal melting in 14.2%. Other complications observed included infectious ulcers, limbal graft necrosis or ischemia, perforation, and descemetocele.Conclusion:Chemical burns remain the main cause of limbal stem cell deficiency. In these cases, limbal transplantation is the standard procedure to restore the ocular surface even though the success rate is low.

RESUMOObjetivo:Análise epidemiológica dos transplantes de limbo realizados no Hospital Oftalmológico de Sorocaba.Método:Foi realizada uma análise retrospectiva através de revisão de prontuário de trinta casos de transplante de limbo realizados no período de janeiro de 2003 a março de 2008. Casos de transplante de limbo autólogo foram classificados como grupo I e alogênicos como grupo II.Resultados:Dois pacientes foram excluídos da análise por insuficiência de dados nos registros. Da amostra completa de 28 pacientes, 53,6% constituíram o grupo I enquanto 46,4% o grupo II. O olho direito (68%) e o sexo masculino (68%) foram mais acometidos, com uma média de idade de 40,3 anos. Casos unilaterais contabilizaram 60,7%. A patologia de base causadora da deficiência límbica mais prevalente foi a queimadura química (53%). A média do tempo de doença até a cirurgia foi de 11,18 anos. Na maioria dos casos o transplante foi associado a membrana amniótica (75%) e tarsorrafia (57%). O tempo médio de seguimento foi de 24,84 meses. Foi observado melhora da acuidade visual não corrigida em 38% dos casos enquanto 28,5% permaneceram inalteradas e 33,3% pioraram. Evolução sem defeito epitelial persistente ocorreu em 75% dos pacientes. A taxa de conjuntivalização foi semelhante nos 2 grupos (53,3% e 58,3%). Em apenas 38% dos casos houve melhora da transparência. A taxa de sucesso foi de cerca de 28%. A complicação mais prevalente foi defeito epitelial persistente (25%) seguida de melting (14,2%). Outras complicações observadas foram úlceras infecciosas, necrose ou isquemia do enxerto, perfuração e descemetocele.Conclusões:A queimadura química permanece como principal patologia causadora de deficiência límbica. Nestes casos o transplante de limbo é atualmente a técnica de eleição para restauração da superfície ocular, porém com baixa taxa de sucesso.

Comparação entre questionários de qualidade de vida e sua correlação com a evolução clínica de pacientes com psoríase / Comparison of quality of life questionnaires and their correlation with the clinical course of patients with psoriasis

FUNDAMENTO: A psoríase é uma dermatose inflamatória crônica caracterizada por lesões eritemato-descamativas que atingem extensas áreas da pele, comprometendo a qualidade de vida dos pacientes por interferir na sua vida pessoal, no relacionamento social e nas atividades diárias. O tratamento permite um bom controle, mas a impressão do paciente, quase sempre, é pouco valorizada. OBJETIVOS: Avaliar a qualidade de vida de pacientes psoriáticos, verificar se há correlação desta com melhoras clínicas e se há equivalência entre dois questionários de qualidade de vida. MÉTODOS: Foram incluídos no estudo pacientes maiores de 18 anos que estavam no início ou troca de tratamento no ambulatório de psoríase do Hospital de Clínicas da Unicamp. Eles responderam dois questionários de qualidade de vida (Psoriasis Disability Index e Dermatological Life Quality Index), até completar 180 atendimentos, para avaliar se haveria correlação entre os questionários. Receberam também um índice clínico (Índice de Área e de Severidade da Psoríase) no tempo inicial da pesquisa e nos retornos subsequentes. RESULTADOS: A pesquisa foi realizada com 138 pacientes (76 homens, idade média de 50 anos). Observou-se correlação entre os questionários aplicados e o índice clínico, mostrando que alterações clínicas se refletem na qualidade de vida, e que há equivalência entre os questionários. CONCLUSÃO: Constatou-se que a maioria dos pacientes atendidos no ambulatório tem apresentado melhora clínica e da qualidade de vida e que há equivalência entre os questionários de qualidade de vida.

BACKGROUND: Psoriasis is a chronic inflammatory dermatosis characterized by erythematous, scaly lesions covering extensive areas of the skin and negatively affecting patients' quality of life by interfering in their personal life, social relationships and routine activities. Treatment offers reasonable control of the condition; however, little importance is generally given to the patient's impression. OBJECTIVES: To evaluate the quality of life of patients with psoriasis, to verify whether there is a correlation between quality of life and clinical improvement and to compare two quality of life questionnaires and evaluate their equivalence. METHODS: Patients over 18 years of age attending the psoriasis outpatient clinic at the Teaching Hospital of the University of Campinas (UNICAMP), who were either initiating treatment or changing to a new form of treatment, were included in the study. Patients answered two quality of life questionnaires (the Psoriasis Disability Index and the Dermatological Life Quality Index) up to a total of 180 consultations to evaluate whether there is a correlation between the questionnaires. Patients were also rated using a clinical index (Psoriasis Area and Severity Index) at baseline and at follow-up visits. RESULTS: The study involved 138 patients (76 men; mean age 50 years). A correlation was found between the questionnaires and the clinical index, showing that clinical changes are reflected in quality of life. The two questionnaires were found to be equivalent. CONCLUSION: It was found that both clinical condition and quality of life improved in the majority of patients receiving care at this outpatient clinic and that the quality of life questionnaires are equivalent.

Polimorfismos dos genes HLA e regiões promotoras do TNF-'alfa'-238 e -308 como fatores de sucetibilidade a psoriase e gravidade da doença / HLA and TNF-Alpha promoter regions -238 and -308 polymorphisms and marks of susceptibility to psoriasis and severety of the disease

Psoríase é uma dermatose inflamatória crônica, determinada por desregulação do sistema imune e associada a várias comorbidades. O marcador genético mais associado à psoríase em todas as populações é o HLA-CW06. Polimorfismos na região promotora do TNF-a, especialmente a troca de uma guanina por uma adenina nas posições -238 e -308 estão relacionados à alta produção de TNF-a e risco aumentado para psoríase nas populações caucasóides e não em asiáticos. Com o objetivo de determinar se polimorfismos destes genes podem ser fatores de risco para susceptibilidade ou gravidade da doença em pacientes brasileiros, foi realizado um estudo caso-controle com 69 pacientes com psoríase de início até 40 anos, com acompanhamento por dez anos para caracterização de sua evolução clínica em doença leve (grupo I) e grave (grupo II), e 70 indivíduos sadios. Foi feita a identificação dos alelos HLA classe I e II e SNPs da região promotora do TNF-a -238 e -308. Coletaram-se 10 mL de sangue periférico dos indivíduos e se realizou a extração do DNA através do método salting out. O DNA foi amplificado pela reação em cadeia da polimerase (PCR), com primers sequência-específica. As freqüências alélicas e gênicas foram estimadas por contagem direta e a comparação entre as frequências dos grupos foi efetuada por Teste de Fisher (programa GraphPad InStat 3.05). Dois métodos computacionais foram usados...

Psoriasis is an erythematous, scaly inflammatory dermatosis with a complex immunologic basis. The strongest genetic marker for psoriasis is HLA-Cw*06. Polymorphisms in the TNF-a promoter region, especially replacement of guanine with adenine in positions -238 and -308 are related to higher TNF-a production and higher risk for psoriasis in Caucasoid populations, not found in Asians. We did a case-control study of 69 patients with psoriasis type I and 70 controls, characterized clinical progression along 10-years of follow-up in mild or severe disease and determined HLA class I, II and TNF-a SNPs -238 and -308 polymorphisms to demonstrate whether these polymorphisms may be genetic risk for susceptibility to psoriasis or severity of the disease in Brazilians. Peripheral blood (10 ml) was collected. Genomic DNA from both psoriasis patients and controls was isolated using a salting out procedure. Polymorphisms were identified by PCR/SSP. Alleles and genotypes frequencies were compared by Fisher's test (GraphPad InStat 3.05 software). Two computational methods were used to determine the haplotypes of each subject, without taking into account any prior information: (1) the ELB algorithm implemented by the ARLEQUIN 3.1 software and (2) a coalescence based method as...