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Background: Hereditary angioedema (HAE) with C1 inhibitor (C1INH) deficiency is an inherited disease characterized by sudden, recurrent episodes of edema that involve the skin, gastrointestinal tract, respiratory tract, and other organs. Objective: Because it takes a long time from the first symptoms to diagnosis, we aimed to identify HAE in untested first-degree blood relatives among some of our patients with HAE in our outpatient clinic at Hospital Universitario Clementino Fraga (HUCFF), Federal University of Rio de Janeiro. Methods: Untested first-degree relatives of patients with HAE C1INH, even those who were asymptomatic, were identified and invited to participate. Those who agreed to participate answered a specific questionnaire and had a blood sample collected for complement testing. Results: Fifty untested first-degree relatives of 30 index patients with HAE C1INH were identified, and both groups were analyzed. The mean ± standard deviation (SD) age of the index patients group was 37.08 ± 16.56 years (range, 13-73 years), with a high frequency in women (n = 24 [80.0%]). Most of them had severe (n = 23 [76.7%]) and moderate (n = 7 [23.3%]) attacks. None were asymptomatic. The mean ± SD time between the first symptoms and diagnosis was 20.2 ± 11.06 years (range, 0-48 years) in that group. In the first-degree relatives group, 30 new cases of HAE C1INH (60%) were identified. Conclusion: We found that there was a long time between early manifestations and a diagnosis of HAE. First-degree relatives of patients with HAE patients are at risk for having the disease. Sixty percent were newly diagnosed with HAE and with C1INH deficiency in our study. So, screening of family members, including individuals who were asymptomatic, is the key for earlier diagnosis and effective treatment.

BACKGROUND: Chronic urticaria is a debilitating disease that considerably affects health-related quality of life, and the Chronic Urticaria Quality of Life Questionnaire is the only questionnaire specifically designed for its evaluation. OBJECTIVE: To evaluate the quality of life of patients with chronic urticaria, using the Brazilian Portuguese version of the Chronic Urticaria Quality of Life Questionnaire. METHODS: The Chronic Urticaria Quality of Life Questionnaire was self-administered in 112 chronic urticaria patients and disease activity was assessed through the Urticaria Activity Score. Clinical and socio-demographic characteristics of patients were studied, such as: age, sex, etiologic diagnosis of chronic urticaria, duration of disease and Urticaria Activity Score. RESULTS: The population studied was composed 85.72% of women with a mean age of 46 years (18-90), while the median disease duration period was 10 years (3 months-60 years). Regarding the etiologic diagnosis, 48.22% had chronic spontaneous urticaria; 22.32% associated with inducible urticaria, 28.57% with chronic autoimmune urticaria, and 23.21% had physical urticaria alone. Disease activity evaluated using the Urticaria Activity Score was 1.04 ± 1.61 (0-6). The total score for the Chronic Urticaria Quality of Life Questionnaire was 36 (0-100) and dimension I (sleep/mental status/eating) had a greater impact on quality of life. The items with the highest mean scores were nervousness and shame over lesions, while the items with the lowest scores were lip swelling and limitations on sporting activities. CONCLUSIONS: Chronic urticaria compromises patients' quality of life, mainly those with more severe disease or who are diagnosed with chronic autoimmune urticaria.

RESUMO Teste de contato atopico com aeroalergenos: uma ferramenta promissora no diagnostico da dermatite atopica 1 Servico de Alergia, Hospital Sao Zacharias, Rio de Janeiro, RJ. 2 Hospital Geral de Nova Iguacu, Nova Iguacu, RJ. 3 Servico de Imunologia, Hospital Universitario Clementino Fraga Filho, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ. 4 FDA Allergenic. Artigo Original Braz J Allergy Immunol. 2013;1(1):65-70. Atopy patch test with aeroallergens: a promising tool in the diagnosis of atopic dermatitis Sergio Duarte Dortas Junior, MD, MSc1,2, Soloni Afra Pires Levy, MD1, Andrea Huguenim Silva Pires, MD, MSc1, Augusto Tiaqui Abe, MD1, Solange Oliveira Rodrigues Valle, MD, PhD1,3, Vilma Perez Coelho, MD1, Ludwig Ruppert Hahnstadt, MSc4, Alfeu Tavares Franca, MD, PhD1,3 Objetivos: Avaliar a padronizacao do metodo com relacao a concentracao do aeroalergeno, tempo de oclusao, de interpretacao; e determinar a especificidade e a sensibilidade do teste de contato alergico (TCA) em relacao ao teste por puntura e a dosagem de IgE especifica, na verificacao da sensibilizacao a acaros em criancas com dermatite atopica (DA). Metodos: Foram selecionadas 72 criancas com idade entre 2 e 12 anos, acompanhadas no ambulatorio de alergia do Hospital Sao Zacharias. Estas foram submetidas a teste de puntura, dosagem de IgEs especificas e TCA para acaros (Dermatophagoides pteronyssinus, Dermatophagoides farinae e Blomia tropicalis). Os testes foram realizados em 3 grupos: (1) DA com ou sem rinite e asma, (2) Rinite e/ou asma sem DA, (3) Saudaveis (controle). Resultados: No grupo 1, 40% dos pacientes apresentaram reacao positiva. A sensibilidade foi maior nos pacientes com maior tempo de exposicao (48 h e 72 h). No grupo 2, o TCA foi mais especifico que sensivel para todos os extratos, com aumento da sensibilidade quanto maior o tempo de exposicao (72 h). No grupo 3, apenas 8,3% apresentaram positividade a algum aeroalergeno do TCA. Conclusao: O TCA mostrou ter valor diagnostico em relacao as reacoes de fase tardia a acaros (D. pteronyssinus, D. farinae e B. tropicalis), com elevada especificidade. Ele demonstrou ser um teste confiavel quando comparado aos resultados do grupo controle. Descritores: Dermatite atopica, teste de contato atopico, acaros. Objectives: To evaluate the standardization of the atopy patch test (APT) with regard to concentration of aeroallergens, occlusion time, and interpretation, and to determine the specificity and sensitivity of the method in relation to prick test and serum specific IgE determination in the investigation of dust mite sensitization in children with atopic dermatitis (AD). Methods: Seventytwo children, ranging from 2 to 12 years of age, were selected among those receiving care at the allergy outpatient clinic of Sao Zacharias Hospital. Children underwent skin prick testing, specific IgE measurements, and APT for mites (Dermatophagoides pteronyssinus, Dermatophagoides farinae, and Blomia tropicalis). Tests were performed in three groups: (1) AD with or without rhinitis and asthma; (2) rhinitis and/or asthma without AD; (3) healthy individuals (controls). Results: In group 1, 40% of the patients presented positive reactions. Sensitivity was higher in patients with longer exposure times (48 h and 72 h). In group 2, APT was more specific than sensitive for all extracts, with increasing sensitivity associated with longer times of exposure (72 h). In group 3, only 8.3% of the individuals were positive to any aeroallergen on APT. Conclusion: APT seems to have diagnostic value in identifying late phase reactions to mites (D. pteronyssinus, D. farinae, and B. tropicalis), with high specificity, and proved to be a reliable test when compared with the results obtained for controls.

We report the case of a 25-year-old woman with Churg-Strauss syndrome, the symptoms of which had first appeared soon after she began taking oral contraceptive at the age of sixteen. The clinical profile evolved rapidly to severe persistent asthma, nasal polyposis, perennial obstructive rhinitis, eosinophilia (peripheral/tissue) and mononeuritis. Churg-Strauss syndrome is the type of disease that demands early detection, accurate diagnosis, aggressive treatment and periodic monitoring. It should be considered in the differential diagnosis of moderate and severe persistent asthma. The case reported calls attention to possibility that there is a hormonal component and that the disease can present early onset.

Background Angioedema is a highly heterogeneous group of conditions and is characterized by sudden, pronounced swelling of the lower dermis and subcutaneous. Because of its frequent coexistence with urticaria, it is often classified in the same manner as urticaria. However, it also includes categories not associated with urticaria. Angioedema without urticaria is characterized by hereditary and acquired angioedema and histaminergic and nonhistaminergic angioedema. The prevalence of subtypes of angioedema without urticaria was estimated at the Clinical Immunology outpatient service of an University Hospital, in Rio de Janeiro.

Methods We report a case of drug rash induced by levothyroxine and currently oral desensitization. A 31 year-old woman had been diagnosed as Hashimoto’s Thyroiditis. She was having levothyroxine for 9 years when she begun to present bilateral eyelids maculopapular rash. Blood exams and physical examination were normal except for the eyelids rash. As she stopped using levothyroxine, the rash has disappeared. Her endocrinologist prescribed her other brands of levothyroxine, and the patient reported the same reaction.

Background Chronic spontaneous urticaria (CSU) is more common in adults, especially middle-aged women. The condition resolves spontaneously within 6 months in 30 to 55% of patients but can persist for years in others. It has a devastating effect on the quality of life of those who experience it. Although the mechanisms are not fully elucidated, anti IgE recombinant humanized monoclonal (Omalizumab) has been recommended, according to the latest guidelines EAACI/GA2LEN/EDF & WAO, with encouraging results in management of refractory CSU as opposed to the usual and alternative therapies.

Short-term prophylaxis (STP) with plasma derived human C1 inhibitor concentrate (pdhC1-INH) in two pregnant women with hereditary angioedema (HAE): an experience in Rio de Janeiro – Brazil Solange Oliveira Rodrigues Valle, Maria Luiza Oliva Alonso, Sergio Duarte Dortas Junior, Soloni Afra Pires Levy, Ana Paula Ferracciu Coutinho Millet, Alfeu Tavares Franca, Alfeu Tavares Franca, Ana Luiza Ribeiro Bard De Carvalho