Chronic Urticaria in the Real-Life Clinical Practice Setting in Portugal: Baseline Results from the Non-Interventional Multicentre AWARE Study.

INTRODUCTION: There is a paucity of information regarding chronic urticaria patients' care in a real-world setting. The objective of this study was to report and evaluate the baseline characteristics of Portuguese chronic urticaria patients refractory to H1-antihistamines included in the AWARE study. MATERIAL AND METHODS: This is a non-interventional cohort study. Adult patients with a diagnosis of chronic urticaria with symptoms for at least two months, refractory to H1-antihistamines, consulting one of the 10 participating urticaria centers throughout Portugal have been included in the study. Baseline sociodemographic data, medical history, clinical parameters, medication, weekly urticaria activity score, and dermatology quality of life index have been collected. RESULTS: Seventy six patients were included, of which 76.3% were women. The majority of patients had a diagnosis of chronic spontaneous urticaria (88.2%) and 39.5% had angioedema. Around 91.0% of patients were medicated with non-sedative H1-antihistamines and 35.4% with a third line therapy. Median dermatology quality of life index was 5.0 and median weekly urticaria activity score was 13.0. DISCUSSION: The baseline results suggest that patients with chronic urticaria refractory to H1-antihistamines are being under-treated in the real-world setting. CONCLUSION: The AWARE study demonstrates the real impact of chronic urticaria on Portuguese patients refractory to H1-antihistamines treatment, and 30% report a very large or extremely large deleterious effect on their quality of life. The follow-up of these patients will allow evaluating strategies aimed at optimizing disease control.

Introdução: A informação sobre os doentes com urticária crónica em ambiente de vida real é escassa e este estudo teve por objectivo reportar e avaliar as características basais dos doentes portugueses com urticária crónica refractários aos anti-histamínicos H1 incluídos no estudo AWARE. Material e Métodos: Estudo de coorte não intervencional. Foram incluídos doentes adultos com diagnóstico de urticária crónica sintomáticos durante pelo menos dois meses, refratários aos anti-histamínicos H1, seguidos em 10 centros de urticária em Portugal. Foram recolhidos dados basais sociodemográficos, história clínica, parâmetros clínicos, medicação, índice semanal de atividade de urticária e índice de qualidade de vida dermatológico. Resultados: Foram incluídos 76 doentes, dos quais 76,3% mulheres. A maioria dos doentes estava diagnosticado com urticária crónica espontânea (88,2%) e 39,5% apresentavam angioedema. Cerca de 91,0% dos doentes estavam medicados com anti-histamínicos H1 não sedativos e 35,4% com terapêuticas de terceira linha. A mediana do índice de qualidade de vida dermatológico foi 5,0 e a mediana do índice semanal de atividade de urticária foi 13,0. Discussão: Os resultados basais sugerem que os doentes com urticária crónica refratários ao tratamento com anti-histamínicos H1 estão sub-tratados em ambiente de vida real. Conclusão: O estudo AWARE vem demonstrar o real impacto da urticária crónica nos doentes portugueses refratários ao tratamento com anti-histamínicos H1 onde mais de 30% reporta um impacto elevado ou extremamente elevado da doença na sua qualidade de vida. O seguimento destes doentes permitirá avaliar estratégias para otimização do controlo da doença.

[Psoriasis in the era of biologics]. / Psoríase na era dos biológicos.

Chronic plaque psoriasis is an immune-mediated, inflammatory skin disease with a heavy burden on quality of life of patients. Conventional systemic therapies, including cyclosporine, methotrexate, acitretin and photo(chemo)therapy, have proved to be effective, but the risk of toxicity prevents their prolonged and continuous use. Advances in the understanding of psoriasis immunopathogenesis have led to the development of drugs, designed to selectively interfere with the immune mechanisms that induce psoriasis, called biologics. These agents have proven to be a convenient, safe and effective alternative to conventional treatments, and have become an important part of the dermatologist therapeutic armamentarium. This review will focus on the mechanisms of action, guidelines for usage, efficacy data and safety concerns of the main biologics used in Europe for the treatment of moderate to severe plaque psoriasis: etanercept, infliximab, adalimumab. Although efalizumab's marketing authorization, approved since 2003, has been suspended recently (February 2009) across the European Union, it will also be briefly discussed.

Disseminated superficial porokeratosis in a patient with cholangiocarcinoma: a paraneoplastic manifestation?

Porokeratosis refers to a group of hereditary or acquired disorders of epidermal keratinization and is characterized histologically by the presence of a cornoid lamella. The clinical variant referred to as disseminated superficial porokeratosis has been described in the literature in association with immunosuppressive conditions that include organ transplant, infections and immunosuppressive treatments. The association of disseminated superficial porokeratosis with solid organ malignancies has seldom been described, only 5 such cases having been published. The present report refers to a patient with lesions of disseminated superficial porokeratosis of sudden onset shortly before diagnosis of a cholangiocarcinoma.

Poroceratose superficial disseminada num doente com colangiocarcinoma: manifestação paraneoplásica? / Disseminated superficial porokeratosis in a patient with cholangiocarcinoma: a paraneoplastic manifestation?

As poroceratoses compreendem um grupo de doenças da queratinização epidérmica, hereditárias ou adquiridas, caracterizadas histologicamente pela presença de lamela cornoide. A variante clínica designada por poroceratose superficial disseminada tem sido descrita na literatura, associada a estados de imunossupressão, como transplantação de órgãos, terapêuticas imunossupressoras e infecções. A sua associação a neoplasias sólidas foi raramente descrita na literatura, estando publicados apenas 5 casos. Descrevemos o caso clínico de um paciente que desenvolveu, subitamente, lesões de poroceratose superficial disseminada, concomitantemente ao diagnóstico de um colangiocarcinoma.

Porokeratosis refers to a group of hereditary or acquired disorders of epidermal keratinization and is characterized histologically by the presence of a cornoid lamella. The clinical variant referred to as disseminated superficial porokeratosis has been described in the literature in association with immunosuppressive conditions that include organ transplant, infections and immunosuppressive treatments. The association of disseminated superficial porokeratosis with solid organ malignancies has seldom been described, only 5 such cases having been published. The present report refers to a patient with lesions of disseminated superficial porokeratosis of sudden onset shortly before diagnosis of a cholangiocarcinoma.