Correlation between the histopathology of chronic urticaria and its clinical picture

Abstract BACKGROUND: Chronic urticaria is characterized by transient, pruritic lesions of varying sizes, with central pallor and well-defined edges, with disease duration longer than six weeks. Its cellular infiltrate consists of neutrophils, lymphocytes and eosinophils. There is a subgroup of patients with eosinophilic or neutrophilic urticaria, resistant to the treatment with antihistamines, but that respond to a combination of antihistamine with other drugs. OBJECTIVE: To evaluate the present infiltration in chronic urticaria biopsies and correlate it with the clinical disease activity and response to treatment. METHODS: Forty-one patients with chronic urticaria were classified according to the score of severity of the disease, response to treatment and type of perivascular infiltrate. Inflammatory infiltrates were divided in eosinophilic (46.30%), neutrophilic and mixed. RESULTS: An association was found between the eosinophilic infiltrate and clinical scores of greater severity (p = 0.002). CONCLUSION: This association shows that the eosinophilic inflammatory infiltrates denote high clinical activity, which means more severe and exuberant clinical pictures of the disease.

Impact of chronic urticaria on the quality of life of patients followed up at a university hospital

Abstract 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.

Urticaria inducible: serie de casos y revisión de la literatura

Las urticarias inducibles constituyen un grupo heterogéneo de trastornos cutáneos caracterizados por la aparición de habones, prurito o angioedema, que en ocasiones se acompañan de síntomas sistémicos causados por estímulos inocuos para la mayoría de la población, como el frío, el calor, la presión, etc., y que comprometen la calidad de vida de los pacientes. La mayor parte de la literatura médica pertinente proviene de reportes y series de casos, ya que su epidemiología se ha estudiado poco. El objetivo de esta revisión es ofrecer una visión actualizada de la información disponible sobre varios tipos de urticaria inducida, mediante la presentación de un caso clínico ilustrativo y la descripción de los mecanismos fisiopatológicos, las manifestaciones clínicas y el tratamiento de cada condición.

Inducible urticaria is a heterogeneous group of skin disorders characterized by the appearance of wheals, pruritus and/or angioedema, sometimes accompanied by systemic symptoms caused by innocuous stimuli (cold, heat, pressure, etc.). This group of disorders compromises people´s quality of life and most of the literature in this regard comes from case reports and case series since its epidemiology has been poorly studied and some cases are very rare. The aim of this review is to show an up-to-date overview of the available literature for various types of inducible urticarias, always beginning with an illustrative case and then describing their pathophysiological mechanisms, clinical manifestations, and treatment.

Guía Argentina de urticaria y angioedema

Se actualiza el diagnóstico de la urticaria crónica (UC) y los conceptos, definiciones y sugerencias basados en la evidencia para su tratamiento. La urticaria ocurre en al menos 20% de la población en algún momento de la vida. Su etiología difiere en la forma aguda (menos de 6 semanas), y en la crónica. No es posible pronosticar si las formas agudas evolucionarán a UC, ya que todas son agudas al comienzo. La UC ocurre como espontánea (UCE) o inducible (UCI). El diagnóstico es sencillo, pero incluye un minucioso estudio para descartar diagnósticos diferenciales; para UCI son útiles las pruebas de provocación en la caracterización y manejo. Los estudios complementarios se deben limitar y orientar según sospecha clínica. El tratamiento se divide en tres enfoques: evitación, eliminación o tratamiento del estímulo desencadenante o de la causa, y tratamiento farmacológico. Recientemente éste se modificó, con empleo de antihistamínicos de segunda generación como primera línea y aumento de dosis de antihistamínicos H1 no sedantes, hasta 4 veces, como segunda línea. Los antihistamínicos son fundamentales para tratar la UC; sin embargo, un 40% de los pacientes no logra un buen control pese al aumento de dosis y requiere otro medicamento adicional. La evidencia más reciente considera que un grupo de fármacos puede utilizarse como tercera línea en estos casos, para mejorar la calidad de vida y limitar la toxicidad por el uso frecuente o crónico de esteroides sistémicos. Se recomiendan para esta tercera línea solo 3 fármacos: omalizumab, ciclosporina A o antileucotrienos.

This interdisciplinary paper summarizes the news in the diagnosis and treatment of chronic urticaria (CU), and provides concepts, definitions and evidence-based suggestions for its management. Urticaria occurs in at least 20% of the population at some point in their lives. Acute urticaria (less than 6 weeks' duration), differs from CU in its etiology, but the onset of this disease is always acute. CU may occur as spontaneous (SCU) or induced (ICU). The diagnosis is simple, although a careful evaluation is necessary for differential diagnosis. ICU´s diagnosis is mainly clinical, even if provocation tests can be useful. Supplementary studies should be limited and based on the clinical suspicion. Treatment may be divided into three approaches: avoidance, elimination or treatment of the cause, and pharmacological treatment. Recently treatment has been modified with the use of second-generation antihistamines as first-line and increased doses of nonsedating H1 antihistamines, up to 4 times, as second line. Antihistamines are essential to treat CU; however, 40% of patients do not achieve good control despite increased doses and require additional treatment. The most recent evidence indicates a group of drugs to be used as third line in these cases, to improve quality of life and to limit toxicity from frequent or chronic use of systemic steroids. Only 3 drugs are recommended as third line: omalizumab, cyclosporin A or anti-leukotrienes.

Guía argentina de urticaria y angioedema / Argentine guidelines for urticaria and angioedema

This interdisciplinary paper summarizes the news in the diagnosis and treatment of chronic urticaria (CU), and provides concepts, definitions and evidence-based suggestions for its management. Urticaria occurs in at least 20

of the population at some point in their lives. Acute urticaria (less than 6 weeks’ duration), differs from CU in its etiology, but the onset of this disease is always acute. CU may occur as spontaneous (SCU) or induced (ICU). The diagnosis is simple, although a careful evaluation is necessary for differential diagnosis. ICU’s diagnosis is mainly clinical, even if provocation tests can be useful. Supplementary studies should be limited and based on the clinical suspicion. Treatment may be divided into three approaches: avoidance, elimination or treatment of the cause, and pharmacological treatment. Recently treatment has been modified with the use of second-generation antihistamines as first-line and increased doses of nonsedating H1 antihistamines, up to 4 times, as second line. Antihistamines are essential to treat CU; however, 40

of patients do not achieve good control despite increased doses and require additional treatment. The most recent evidence indicates a group of drugs to be used as third line in these cases, to improve quality of life and to limit toxicity from frequent or chronic use of systemic steroids. Only 3 drugs are recommended as third line: omalizumab, cyclosporin A or anti-leukotrienes.

Estudo da qualidade de vida nos pacientes com urticária crônica / Quality of life assessment in patients with chronic urticaria

FUNDAMENTOS: A urticária crônica compromete o doente por interferir nas atividades diárias, prejudicar a autoestima e as relações interpessoais. Os profissionais de saúde subestimam seu impacto na qualidade de vida dos doentes. OBJETIVOS: Avaliar a qualidade de vida com questionário específico e genérico. Compará-la entre os tipos clínicos de urticária crônica e avaliar se o angioedema piora a qualidade de vida. MÉTODOS: Participaram 62 doentes com urticária crônica, com sinais e sintomas da doença até 7 dias da consulta, que foram divididos em urticária crônica comum, urticária crônica física e urticária crônica mista. RESULTADOS: Observou-se predominância de mulheres (72,6 por cento), idade média de 39,8 anos, angioedema associado em 75,8 por cento dos doentes. Apresentaram mais angioedema doentes com urticária crônica comum (p=0,011) e mulheres (p=0,024). Quanto aos tipos clínicos, 32,3 por cento apresentaram urticária crônica comum, 27,4 por cento urticária crônica física e 40,3 por cento urticária crônica mista. O escore médio total do questionário específico foi 10,4. No questionário específico, os domínios mais comprometidos foram "Sintomas e sentimentos" e "Atividades diárias", e, no SF-36, "Aspectos físicos" e "Vitalidade". Houve comprometimento da qualidade de vida nas mulheres, nos doentes com até 30 anos, em primeira consulta, nos mais escolarizados, naqueles com até 1 ano de doença e naqueles com angioedema. CONCLUSÃO: A urticária crônica compromete a qualidade de vida medida pelos questionários específico e genérico. Não houve diferença estatisticamente significante na qualidade de vida entre os tipos clínicos. A presença do angioedema conferiu pior qualidade de vida aos doentes. Houve correlação estatisticamente significante entre os escores do questionário específico e do questionário genérico.

BACKGROUND: Chronic urticaria affects patients by interfering with their daily activities, damaging their self-esteem and negatively affecting their interpersonal relationships. Healthcare professionals may underestimate the impact of the condition on patients' quality of life. OBJECTIVES: To evaluate quality of life using one generic and one disease-specific health-related quality of life instrument; to compare quality of life between the different clinical types of chronic urticaria and to evaluate whether angioedema further impairs quality of life. METHODS: Sixty-two patients with chronic urticaria, who had had signs or symptoms of the disease within the preceding week, were divided into groups according to whether they had ordinary chronic urticaria, physical chronic urticaria or mixed chronic urticaria. RESULTS: There was a predominance of women in this study (72.6 percent). The mean age of patients was 39.8 years and angioedema was associated with chronic urticaria in 75.8 percent of cases. Associated angioedema was more commonly found in patients with ordinary chronic urticaria (p=0.011) and in women (p=0.024). With respect to the different clinical types, 32.3 percent of the patients had ordinary chronic urticaria, 27.4 percent had physical chronic urticaria and 40.3 percent had mixed chronic urticaria. Mean overall score for the disease-specific questionnaire was 10.4. In this questionnaire, the most affected domains were "symptoms and feelings" and "daily activities", while the most affected domains in the SF-36 were "physical role" and "vitality". Quality of life was found to be impaired in women, in patients of up to 30 years of age, in those attending a first consultation, in those with higher education levels, in patients who had had the disease for up to one year and in those with angioedema. CONCLUSION: Chronic urticaria affects quality of life, as measured using a disease-specific questionnaire and a generic questionnaire. There was no statistically significant difference between the clinical types. The presence of angioedema impaired patients' quality of life even further. There was a statistically significant correlation between the scores obtained using the disease-specific questionnaire and those obtained using the generic questionnaire.

Urticaria crónica autoinmunitaria: tratamiento con omalizumab / Chronic autoimmune urticaria: treatment with omalizumab

Se presenta el caso de una niña con diagnóstico de urticaria autoinmunitaria y su evolución terapéutica con omalizumab. Caso clínico. Paciente de sexo femenino de 12 años de edad con un cuadro de urticaria crónica grave y angioedema de 14 meses de evolución, escasa respuesta a dosis máximas de 3 antihistamínicos combinados, antileucotrienos y corticoides, y gran afectación de su calidad de vida. Se realizó una prueba de suero autólogo, que fue positiva hasta la dilución 1:100, llegándose al diagnóstico de urticaria crónica autoinmunitaria. La falta de respuesta al tratamiento lleva a indicar terapia con omalizumab, con una reducción notable de los síntomas hacia la tercera dosis y ausencia de ellos tras 12 meses de tratamiento. Conclusión. El omalizumab podría ser una opción terapéutica para pacientes con urticaria autoinmunitaria que no responden a otros tratamientos.

We report the case of a child with diagnosis of chronic urticaria/angioedema and its evolution upon omalizumab treatment. Case report. Our patient is a 12-years-old female who suffered for 14 months severe chronic urticaria/angioedema. She had a poor response to the highest doses of combined therapy with 3 antihistamines, steroids and anti-leukotrienes and great impairmentof her quality of life. An autologous serum skin test was positive until 1:100 dilutions, leading to the diagnosis of chronic autoimmune urticaria. Due to the lack of response to treatment, therapy with omalizumab was administered. A notable reduction in symptoms toward the third dose was observed. After 12 months of this treatment, the patient is asymptomatic and has a negative autologousserum test. Conclusion. Omalizumab could be a therapeutic option for patients with autoimmune urticaria unresponsive to other treatments.

Prurito cutaneo / Cutaneous pruritus

Concepto: El prurito (de prurio: picor, picazón, comezón) es un síntoma subjetivo y de difícil definición, sin embargo también es de fácil compresión, pues con seguridad todos los individuos lo han experimentado alguna vez en la vida. La definición clásica del prurito es: ‘sensación desagradable que conduce al rascado’. Es un hecho fisiológico que surge ante estímulos externos o internos en forma súbita y autolimitada y que provoca como repuesta el rascado. No obstante, el prurito también puedes ser patológico y acompañarse de diversas enfermedades cutáneas o sistémicas. El prurito es un síntoma aflictivo tan difícil de soportar como el dolor, ya que puede deteriorar la calidad de vida desde el punto de vista psíquico y psicológico...

Urticaria crónica / Chronic Urticaria

La urticaria es una condición heterogénea que puede manifestarse clínicamente de formas muy diversas, cuyo signo más común es el habón o roncha. Existen muchas posibles causas de urticaria, y la gravedad y patrón clínico pueden variar considerablemente de paciente a paciente. Por este motivo el tratamiento debe ser individualizado y debe modificarse caso a caso. Clínicamente consiste en una erupción temporal de zonas eritematosas y edematosas que corresponden a aumentos de volumen de la dermis, generalmente acompañados de prurito. La urticaria es clasificada en aguda y crónica según su duración, considerándose seis semanas como el tiempo máximo para hablar de urticaria aguda, cuando las lesiones están presentes por más de seis semanas, hablamos de urticaria crónica (UC). Existen múltiples factores gatillantes de urticaria, como alimentos, inhalantes, fármacos, infecciones, etc. El tratamiento es complejo y consiste en medidas no farmacológicas (ambientales), las que pueden llevarse a cabo más fácilmente en la medida que se identifiquen los factores gatillantes, y en el uso de fármacos principalmente antagonistas anti H1. En este artículo se discutirá sobre los aspectos generales de la urticaria crónica, los tratamientos que hoy existen y aquellos que se están desarrollando.

Ordinary urticaria: a descriptive study

Clinical and histopathological evaluation of acute and chronic ordinary urticaria. Eighty-two patients having ordinary urticaria, 57 [69.5%] acute and 25 [30.5%] chronic, 24 [29.27%] males and 58 [70.73%] females, had been recorded from the out patient clinic-department of dermatoIog and venerolog-Baghdad Teaching Hospital-Baghdad. Data abstracted from the patients by a questionnaire and clinical assessment. All patients were sent for laboratory investigations including, blood examination, general urine and stool examination. Antinuclear factor was carried out for those patients with chronic urticaria. The mean age of the patients was 24 +/- 4.6 years. Females were more affected than males. Regarding atopy, there was no much difference from control. Night exacerbation was noticed in ordinary urticaria 43 [52.4%]. Emotional tension 32 [39%], food 25 [30.5%], heat 25 [30.5%]. drugs 16 [19.5%] and infections 14 [17%] were the most provoking factors. Gastrointestinal disturbances were the most common associated symptoms and noticed in 23.2% of patients. These symptoms were more in acute urticaria [29.8%] when compared with chronic urticaria [8%]. Parasitic infestations were noticed in chronic urticaria [20.4%], while eosinophilia was more evident in acute urticaria [16.1%]. Positive ANF, leukocytosis, high ESR, anemia and pyuria were observed in some patients. Tuberculin testing was less positive in patients with urticaria. Acute and chronic ordinary urticaria constitute a common type of urticaria affect predominantly females. Good questionnaire and full investigations are essential for the detection of their provoking factors

Urticária- angioedema crônico: caracterização sociodemográfica, clinica e etiológica - Estudo em doentes atendidos no HC - FMB - UNESP no período de 1990 a 1998 / Urticaria - chronic angioedema: sociodemographic, clinical and etiological characterization - Study in patients attended at HC - FMB - UNESP from 1990 to 1998

Urticaria cronica-angioedema e uma enfermidade complexa, multicausal, de facil diagnostico clinico, representando cerca de 1,1% das consultas ambulatorial dermatologicas do H.C. da F.M.B., no periodo de 1990 a 1998. Os objetivos deste estudo visaram caracterizar a populacao atendida quanto aos aspectos sociodemograficos, clinicos e etiologicos, assim como o estudo de fatores agravantes e a evolucao da enfermidade nos doentes atendidos no referido periodo. Para os objetivos propostos, foram avaliados 125 doentes atraves de um protocolo previamente elaborado e constava de: identificacao, anamnese dirigida, exame dermatologico, fisico e subsidiario. Na avaliacao dos resultados, observou-se predominancia no sexo feminino, no grupo etario de 20 a 50 anos, principalmente na faixa dos 30 a 40 anos, com idade media de 35 anos para o sexo feminino e 32 para o masculino. Houve predominancia da cor branca e em moradores da zona urbana. Nao houve ocupacao prevelente e cerca de 60% eram casados. Com relacao aos aspectos clinicos, o tempo medio de duracao da enfermidade foi de 45,26 meses, as lesoes tiveram duracao media de 5,6 horas e o surto 6,5 horas. Nao houve horario preferencial para o aparecimento dos surtos e o periodo noturno foi o mais citado para piorar o quadro ja estabelecido. Nao houve fatores associados na maioria dos casos e o estresse foi o fator agravante mais citado. Na metade dos doentes houve associacao com angioedema e o quadro clinico ocorria diariamente, seguido de reaparecimento a intervalos irregulares. A renite foi o antecedente alergico pessoal mais citado e a bronquite a antecedente alergico familiar. A maioria dos doentes nao apresentavam urticaria na 1ª consulta ambulatorial e em 37% havia outra enfermidade associada. Entre outras doencas associadas, as alteracoes no aparelho genito-urinario, digestivo e respiratorio foram os mais citados, seguidos das doencas endocrinas, nutricionais ou metabolicas, transtornos circulatorios e mentais. Ao c...

Aspectos etiopatogénicos de la urticaria y el angioedema en la consulta alergológica / Etiopathogenic aspects of the urticaria and the angioedema in the alergologic consultation

El objetivo de este estudio fue conocer la incidencia de urticaria y angioedema en la consulta alergológica. Se determinó que proporción de los pacientes fueron alérgicos y se trató de conocer la etiología de los cuadros clínicos. Se analizaron en forma retrospectiva las historias clínicas de los pacientes que consultaron por primera vez en un centro privado de alergia e inmunología en el lapso de doce meses. Se excluyeron los pacientes que consultaron exclusivamente por pruebas de provocación a medicamentos. De 232 pacientes, 46 presentaron urticaria y angioedema. La forma de presentación más frecuente fue el angioedema (41,3 por ciento), segundo la urticaria (32,6 por ciento) y por último ambas enfermedades asociadas (26,1 por ciento). Veinticinco pacientes refirieron antecedentes familiares de alergia (54,3 por ciento). Sobre 39 pacientes 22 presentaron un IgE sérica total mayor de 100Ul/ml. Sólo 7 pacientes presentaron eosinofilia (> 350 mm3). En conclusión, la urticaria y el angioedema son motivo de consulta frecuente en alergia con una incidencia de 19,8 por ciento en un año. La mayoría de los pacientes (78,2 por ciento) presentó antecedentes familiares alérgicos y/o IgE sérica total elevada. Medicamentos, alimentos, ácaros y fenómenos físicos fueron factores desencadenantes de los cuadros clínicos