Episodic angioedema with eosinophilia (Gleich's syndrome) associated with urticarial vasculitis: a coincidence or a novel clinical entity?

Episodic angioedema with eosinophilia (EAE) is a rare condition characterized by recurrent attacks of angioedema and urticaria accompanied by a marked elevation of peripheral eosinophil count. We report the case of a young female patient diagnosed with EAE associated with urticarial vasculitis. A 40-year-old female patient was admitted to our institution due to recurrent episodes of cheek and eyelid angioedema in the previous year. Episodes of facial angioedema lasted for two months with spontaneous remission afterwards. In addition, she presented pruritic and painful skin eruptions of erythematous circles, which persisted for longer than 24 h, that were palpable, somewhat purplish, and more pronounced on the face, arms, and trunk. Laboratory investigation showed a sustained elevation of white cell counts with marked eosinophilia. Serum IgM, IgE, and IgA were normal; IgG was slightly elevated. C1-esterase inhibitor and tryptase test were normal. Reverse transcriptase-polymerase chain reaction was performed for detection of FIP1L1-PDGFRA and BCR-ABL rearrangements. None of these alterations were found. Skin biopsies were suggestive of urticarial vasculitis. The patient was submitted to esophagogastroduodenoscopy, which showed mild chronic gastritis, with no eosinophilic infiltration. Cardiac dimensions and function were normal. Abdominal ultrasound and total body CT-scan failed to show lymphadenopathy, organomegaly, and tumors. We report the first case of association between episodic angioedema with eosinophilia and urticarial vasculitis. It is possible that both conditions share a physiopathological mechanism, suggesting that it is not just a chance association.

A Case of Schnitzler's Syndrome without Monoclonal Gammopathy-Associated Chronic Urticaria Treated with Anakinra

Chronic urticaria may often be associated with interleukin (IL)-1-mediated autoinflammatory disease, which should be suspected if systemic inflammation signs are present. Here, we report a case of Schnitzler's syndrome without monoclonal gammopathy treated successfully with the IL-1 receptor antagonist anakinra. A 69-year-old man suffered from a pruritic urticarial rash for 12 years. It became aggravated episodically and was accompanied by high fever, arthralgia, leukocytosis, and an elevated C-reactive protein and erythrocyte sedimentation rate. The episodes each lasted for over one week. Neutrophilic and eosinophilic inflammation was found on skin biopsy. However, serum and urine electrophoresis showed no evidence of monoclonal gammopathy. The cutaneous lesions were unresponsive to various kinds of anti-histamines, systemic glucocorticoids, colchicine, cyclosporine, dapsone, and methotrexate, which were administered over a span of 3 years immediately preceding successful treatment. A dramatic response, however, was observed after a daily administration of anakinra. This observation suggests that the correct diagnosis of this case is Schnitzler's syndrome without monoclonal gammopathy. For an adult patient with refractory chronic urticaria and systemic inflammation, Schnitzler's syndrome could be considered as a possible differential diagnosis. Although the typical form of Schnitzler's syndrome exhibits the presence of monoclonal gammopathy as a diagnostic criterion, monoclonal gammopathy may be absent in an atypical form. In such a situation, an IL-1 antagonist should be effective for the management of chronic urticaria.

Dermografismo "mixto o bifásico" / "Mixed or biphasic" Dermographism

El dermografismo, conocido como "escritura sobre la piel", fenómeno que ocurre en forma espontánea o a la provocación física de la misma, es un síntoma, signo o síndrome semiológico, característico de procesos clínicos alérgicos y no alérgicos. Se expresa comúnmente como dermografismo rojo (urticaria dermatográfica, con o sin angioedema acompañante), con eritema y/o roncha muy frecuente en procesos alérgicos, pero también en las urticarias físicas, autoinmunes o tóxicas por aditivos alimentarios, infecciones, medicamentos y otros agentes. El dermografismo blanco, que se evidencia como un área que palidece alrededor de la línea de estimulación física, muy característico de la atopia; y, el que en esta comunicación hemos denominado dermografismo "mixto o bifásico", por su carácter bimodal, en el que se alternan las expresiones de los dermografismos rojo y blanco, en forma casi simultánea o en sucesión de blanco temprano en el primer minuto a rojo tardío a los cinco minutos o más, o al contrario, rojo inmediato y blanco posterior, típico de atopia. El paciente con hiperreactividad atópica en piel es el que exhibe el dermografismo mixto o bifásico, pues muestra el dermografismo blanco como estigma de atopia y el dermografismo rojo de la urticaria sintomática aguda o crónica. Esta última forma de dermografismo no se encuentra en las descripciones clásicas y es el objetivo de esta comunicación, que se acompaña de una amplia discusión sobre la ocurrencia de dermografismo en la práctica clínica. Material y Métodos: Se realizó una extensa revisión bibliográfica, consultando bases de datos como Medline, PubMed, DocChek, Wiley, Amedeo, Cochrane, Scielo, Hinari y Lilacs; se presentan viñetas clínicas de casos de pacientes atópicos. Conclusión: En esta revisión se ha presentado la ocurrencia clínica del dermografismo como un fenómeno frecuente de diferentes orígenes, pero que, muchas veces, es acompañante casi invariable de expresiones atópicas, en donde el dermografismo es un signo de ayuda para la tipificación del paciente alérgico...(AU)

Allergic reaction to patent blue dye in breast surgery - case report / Reação alérgica ao corante azul patente em cirurgia de mama - relato de caso

Abstract We present a case of allergic reaction to patent blue in a patient who underwent excision of sentinel lymph node associated with segmental breast resection. About 20 min after the dye injection, the patient developed hypotension (BP = 70 × 30 mmHg) associated with increased heart frequency. The patient was treated successfully with decreased inspired fraction of inhaled anesthetic and fluid replacement. At the end of the procedure, she presented with bluish urticarial-like plaques on the head, neck, upper limbs, and trunk; hydrocortisone was then used. The patient recovered uneventfully and was discharged from the PACU 2 h after the end of surgery without skin changes, and was discharged from hospital on the morning after surgery. The incidence of allergic reactions with the use of patent blue is far superior to the hypersensitivity reactions seen with anesthetic and adjuvant drugs. Therefore, the anesthesiologist must be aware of cardiovascular instability associated with skin changes during the use of patent blue, for early diagnosis and appropriate treatment of this hypersensitivity reaction to this dye.

Resumo Os autores apresentam um caso de reação alérgica ao azul patente em uma paciente submetida à exérese de linfonodo em sentinela associada a uma ressecção segmentar de mama. Paciente apresentou aproximadamente pós 20 minutos da injeção do corante hipotensão (PA = 70 × 30 mmHg) associada a aumento da frequência cardíaca. Foi tratada satisfatoriamente com diminuição da fração inspirada do anestésico inalatório e reposição volêmica. No fim do procedimento apresentava placas urticariformes azuladas em cabeça, pescoço, membros superiores e tronco e foi usada hidrocortisona. Evoluiu, sem intercorrências, na sala de recuperação pós-anestésica e teve alta duas horas após o término do procedimento cirúrgico sem a presença das alterações cutâneas. Alta hospitalar na manhã seguinte à cirurgia. A incidência de reações alérgicas com o emprego do azul patente é muito superior às reações de hipersensibilidade observadas com drogas anestésicas e adjuvantes. Portanto, o anestesiologista deve ficar atento à instabilidade cardiovascular associada a alterações cutâneas quando do uso do azul patente para o diagnóstico precoce e tratamento adequado dessa reação de hipersensibilidade com o emprego do corante.

Incidence of allergic contact sensitization in central Chinese subjects with chronic urticaria

Abstract BACKGROUND: Chronic urticaria (CU) can be provoked by a wide variety of causes. Some studies suggest contact sensitization may play a role in the disease. OBJECTIVE: To investigate the incidence and distribution characteristics of allergic contact sensitization in central Chinese subjects with CU, and assess contact allergen avoidance measures in managing CU. METHODS: Patch tests were performed, following the recommended standard procedure, with 20 selected allergens, in line with the European baseline series. All subjects with positive results were prescribed appropriate avoidance measures for the sensitizing substances, while subjects with negative results served as the control group. CU severity was assessed daily from week1 to week4 and for each subject, applying the Urticaria Activity Score. RESULTS: 42.9% (233/543) of subjects with CU showed positive reactions to one or more contact allergen(s). Potassium dichromate, benzene mix and carba mix were more common in male patients, while nickel sulfate was more frequent in females. The positive rates for different allergens varied with age and occupation. The median (interquartile range) severity scores at week 1 were 20 (14-21) and 15 (14-27) for the allergen avoidance group and control group, respectively (P>0.05); and 12 (7-15) and 14 (12-17) at week 4 (P<0.001). CONCLUSION: The incidence of allergic contact sensitization in CU patients was high, and appropriate contact allergen avoidance measures benefitted CU management. Contact allergens may play a role in the pathogenic mechanism of CU and patch tests are an option for CU patients.

Is There an Association between Restless Legs Syndrome and Urticaria?

Restless legs syndrome (RLS) is a disease characterized by the urge to move the legs and sleep disturbances. Similarly, chronic spontaneous urticaria (CSU) is a dermatological disease characterized by pruritus and sleep disorders. In this study, we aimed to determine the prevalence and severity of RLS in patients with chronic spontaneous urticaria (CSU) and to compare the quality of sleep of patients with and without RLS in the CSU group using the Pittsburgh Sleep Quality Index. A total of 130 patients with CSU and 100 healthy controls were included in this study. The frequency of RLS, frequency of sleep disturbances, and average score of RLS in patients with CSU were statistically significantly higher than control groups (respectively P = 0.008, P = 0.009, P = 0.004). Subjective sleep quality, sleep latency and habitual sleep efficiency scores in patients with RLS were statistically significantly higher than patients without RLS (respectively P = 0.016, P = 0.007, P = 0.035). We claimed that pruritus of urticaria may decrease the quality of sleep in patients with RLS and it may trigger and worsen the restless legs syndrome. Furthermore, RLS and CSU may share a common etiology.

Urticária crônica e comorbidades mais frequentemente relacionadas / Chronic urticaria and more frequently related comorbidities

Objetivo: Avaliar a associação entre urticária crônica e doenças sistêmicas (síndrome metabólica, artropatias, doença vascular periférica e do trato gastrintestinal). Métodos: Estudo transversal, descritivo, de base clínica. Foi realizado um levantamento de dados por meio de prontuários de 95 pacientes, que estiveram em tratamento ambulatorial de rotina de abril de 2014 a abril de 2015, mediante assinatura de um Termo de Consentimento Livre e Esclarecido pelo paciente durante suas consultas de rotina no Ambulatório de Urticária. Para analisar a associação das doenças, foi utilizado o teste estatístico qui quadrado, considerando sexo feminino e masculino, e a doença. Consideraram- se relevância estatística as relações com p abaixo de 0,05 (p<0,05). O nível de significância adotado foi de 95%. O programa utilizado foi o Stata 11.0. Resultados: Artropatias foram positivas em aproximadamente 55% das mulheres do estudo e 17% dos homens (p=0,001 e p<0,05, respectivamente). Houve significância estatística na relação entre doença vascular e urticária crônica (p=0,022 e p<0,05, respectivamente). Conclusão: Não foi encontrada associação relevante estatisticamente para afirmar que houve confiança na associação entre diabetes e urticária crônica (p=0,801 e p>0,05, respectivamente). Não se obteve relevância na relação com doenças do trato gastrointestinal (p=0,437/p>0,05). relevância na relação com doenças do trato gastrointestinal (p=0,437/p>0,05).

Objective: To evaluate the association between Chronic urticaria and systemic diseases such as metabolic syndrome, arthropathies, peripheral vascular disease, and gastrointestinal diseases. Methods: a cross-sectional, descriptive study of clinical basis. A survey of data was performed through the medical records of 95 patients who underwent routine outpatient treatment from April 2014 to April 2015, and signed na Informed Consent during their routine appointments in the Urticaria Clinic. To analyze the diseases associations we used the statistical Chi-square test, considering male and female patients, and the disease. Relations with p lower than 0.05 (p<0.05) were considered statistically relevant. Significance level was 95%. The software used was Stata 11.0. Results: Arthropathies were positive in approximately 55% of women in the study, and 17% of men (p=0.001 and p<0.05, respectively).There was a statistically significant relationship between vascular disease and chronic urticaria (p=0.022 and p<0.05, respectively). Conclusion: No statistically significant association was found to state that there was confidence in the association between diabetes and Chronic urticaria (p=0.801 and p>0.05, respectively). No relevant relation was observed with gastrointestinal diseases (p=0.437/ p>0.05).

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.

Omalizumab: opción terapéutica para la urticaria crónica espontánea de difícil control con vasculitis asociada, reporte de tres casos / Omalizumab: therapeutic option in chronic spontaneous urticaria difficult to control with associated vasculitis, report of three cases

Introducción. Aproximadamente el 50 % de los casos de urticaria crónica no mejoran adecuadamente con las dosis convencionales de antihistamínicos, por lo cual se han planteado múltiples opciones terapéuticas, entre las cuales el omalizumab es una herramienta novedosa que ahora cuenta con evidencia de alta calidad que soporta su uso en los casos difíciles, que mejora rápidamente el índice sintomático y el uso de medicamentos, y cuenta con un buen perfil de seguridad. Objetivo. Presentar tres casos de mujeres adultas con urticaria crónica espontánea de más de ocho años de evolución, que no mejoraron con el tratamiento con altas dosis de antihistamínicos, asociados a antileucotrienos e inmunomoduladores y en quienes se combinaban varios mecanismos fisiopatológicos: urticaria crónica espontánea con componente de autoinmunidad, componente de presión y urticaria vasculítica. Materiales y métodos. Se reportan los casos con sus respectivas evaluaciones clínicas y de laboratorio, los medicamentos usados y la respuesta después del inicio de omalizumab y se hace una revisión de la literatura científica sobre uso de este medicamento en la urticaria crónica. Resultados. En los tres casos presentados se obtuvo una mejoría completa de los síntomas tras el inicio del omalizumab. Conclusión. El omalizumab es una opción terapéutica exitosa en casos de urticaria crónica de difícil control con vasculitis asociada, cuando se han agotado las opciones propuestas por las guías internacionales.

Introduction: Approximately 50% of chronic urticaria cases do not respond adequately to conventional doses of antihistamines, so a number of other therapeutic options have been suggested. Among these, omalizumab is an innovative tool, which now has high-quality evidence that supports its use in difficult cases, rapidly improving the symptom index and the use of medications with a good safety profile. Objective: To report three cases of adult women with spontaneous chronic urticaria with an evolution of more than eight years, which did not improve with high doses of antihistamines and leukotriene receptor blockers, associated with immunomodulatory therapy in which several etiologic mechanisms were combined: chronic spontaneous urticaria with autoimmune and pressure components, and vasculitis. Materials and methods: We report the cases with their clinical and laboratory evaluations, used medication, the response after the start of omalizumab and we performed a review of the literature on the use of this drug in chronic urticaria. Results: In all the presented cases, we obtained complete improvement of symptoms after starting omalizumab. Conclusion: Omalizumab is a successful treatment option in cases of difficult to control chronic urticaria with associated vasculitis in which the options proposed by international guidelines have been exhausted.

The unusual association of Graves' disease, chronic spontaneous urticaria, and premature ovarian failure: report of a case and HLA haplotype characterization / A associação incomum entre doença de Graves, urticária crônica idiopática e insuficiência ovariana prematura: relato de caso clínico e tipagem HLA

Chronic spontaneous urticaria (CSU), defined as the occurrence of spontaneous wheals for more than six weeks, has been associated with autoimmune diseases. Herein, we report the unusual association of CSU, Graves' disease, and premature ovarian failure. Human leukocyte antigen (HLA) studies were performed. A 36-year-old woman presented symptoms and signs of hyperthyroidism for three months. In the same period, the patient complained of widespread urticarial wheals, intensely itchy, and poorly responsive to therapy with antihistaminic agents. Hyperthyroidism was confirmed biochemically, and treatment with methimazole was started. As hyperthyroidism improved, a marked improvement in her urticaria was also observed. However, the patient continued to complain of amenorrhea. Endocrine evaluation, at the age 38, was consistent with premature ovarian failure. This is the first report of coexistence of GD, CSU, and POF. The genetic background of such unusual association is a specific combination of HLA.

A urticária crônica idiopática, caracterizada pelo aparecimento de pápulas espontâneas e persistentes por pelo menos seis semanas, tem sido associada a doenças autoimunes. Apresentamos aqui o caso da associação incomum entre urticária crônica idiopática, doença de Graves e falência ovariana prematura. Foram conduzidos estudos de tipagem HLA. Uma mulher de 36 anos apresentou sinais e sintomas de hipertireoidismo por três meses. No mesmo período, a paciente queixou-se do aparecimento de pápulas urticariformes generalizadas que coçavam intensamente e não eram responsivas ao tratamento com anti-histamínicos. O hipertireoidismo foi confirmado bioquimicamente, e o tratamento com metimazol foi iniciado. Assim que os valores hormonais se normalizaram, observou-se uma melhoria significativa do quadro de urticária. No entanto, a paciente continuou a apresentar amenorreia. A avaliação endocrinológica, com a idade de 38 anos, mostrou falência ovariana prematura. Este é o primeiro caso de associação entre doença de Graves, urticária idiopática crônica e falência ovariana prematura. A base genética dessa associação incomum é representada por combinações específicas de haplótipos HLA.

Evaluation of itching sensation in chronic urticaria

Itching is the main symptom in urticaria and can cause a lot of suffering in chronic urticaria. To measure the itch threshold in patients with chronic urticaria before and after treatment and compare them with healthy control. The study was conducted at the department of dermatology, medical city teaching hospital Baghdad in the period from December 2009 to July 2010. A total of 30 patients with chronic urticaria [patients group] and 25 healthy individuals [control group], were included in the study. The age of patients ranged between 21years and 48 years with a mean of 30.96 years +/- 6.09 years [standard deviation]. In control group the age ranged between 20 years and 45 years with a mean of 31.40 years +/- 8.39 years [standard deviation]. Regarding gender, in the patients group 19 were females and 11 were males. While in the control group 14 were females and 11 were males. Electrical skin itch threshold was measured by square wave DC electrical stimulator at 13 points on the skin surface in patients and compared to the healthy controls. Doxepin was given to patients for 2 weeks, the itch threshold was then measured again. The severity of urticaria was also assessed before and after treatment. Patients with chronic urticaria had significantly lower threshold than the control subjects .The lowest threshold recorded in patients group was [1.292 +/- 0.399] volts in the left cheek, while in control group the lowest electrical cutaneous threshold was recorded in the chin [1.89 +/- 0.490] volts then left cheek [1.910 +/- 0.478]. The P-value was significance in all skin spots. The electrical cutaneous threshold increased after treatment in all spots tested after treatment. The increase was statistically significant in all tested the skin spots except in the back of the neck and xyphoid point. The total score decreased from 10.8 before treatment with doxepin to 1.5 after treatment. The individual parameters also decreased significantly after treatment. There were no significant side effects except drowsiness which improved after a few days even with continuation of treatment. Electrical skin itch threshold seems to be a simple test that aids in measuring the severity of itching in urticaria and may be used in the evaluation of drugs used for the alleviation of itching in urticaria and other skin diseases.

Vasculite urticariforme com comprometimento renal glomerular: relato de caso / Urtical vasculitis with renal glomerular disease: case report

JUSTIFICATIVA E OBJETIVOS: A vasculite urticariforme (VU) corresponde entre 5% a 10% das urticárias crônicas, devendo-se distinguir suas lesões daquelas da urticária crônica idiopática, que é a forma mais comum. Apesar de comumente ser de origem idiopática, pode ocorrer em associação com doenças autoimunes, reação a drogas, infecções ou malignidade, podendo ocorrer de forma sistêmica ou limitada à pele. O diagnóstico de VU deve ser considerado na presença de urticária persistente com achados clínicos e sorológicos sugestivos, ou evidência de doença sistêmica. O objetivo deste estudo foi mostrar um caso raro de acometimento renal da forma normocomplementêmica da vasculite urticariforme. RELATO DO CASO: Paciente do sexo masculino, 38 anos, previamente hígido, que apresentava lesões papulares eritêmato-violáceas extensas. Exames laboratoriais iniciais não apresentavam alterações significativas; exame qualitativo de urina demonstrava alteração progressiva da função renal e níveis nefróticos de proteinúria. A imunofluorescência renal foi negativa. Sorologias foram todas negativas e exames imunológicos não reagentes. Dosagemde complemento sérico (C3 e C4) foi normal. Realizou-se pulsoterapia com corticoide endovenoso e seguimento com corticoide oral, obtendo-se boa resposta clínica. A lesão cutânea apresentou regressão espontânea sem o uso de medicação tópica. CONCLUSÃO: O diagnóstico da doença sistêmica a partir de uma alteração cutânea salienta a importância da investigação adicional das lesões vasculares de pele.

BACKGROUND AND OBJECTIVES: Urticarial vasculitis (UV) corresponds between 5% to 10% of chronic urticaria, and their lesions must be distinguished from those of chronic idiopathic urticaria, which is more common. Although commonly idiopathic, it may occur associated with autoimmune diseases, drug reactions, infections or malignancy, which may occur in asystemic presentation or limited to the skin. The diagnosis of UV should be considered in the presence of persistent urticaria with suggestive clinical and serologic findings, or evidence of systemic disease. The case report illustrates a rare renal complication of the normocomplementemic form of urtical vasculitis. CASE REPORT: Male patient, 38 years, previously healthy, who presented extensive erythematous-violaceous papules. Initial laboratory tests showed no significant changes, qualitative urine test showed progressive impairment of renal function and levels of nephrotic proteinuria. Renal immunofluorescence was negative. Serology and immunological tests were all negative. Dosage of serum complement (C3 and C4) was normal. We performed intravenous steroid pulse therapy and follow up with oral steroids,obtaining good clinical response. The skin lesions regressed spontaneously without the use of topical medication. CONCLUSION: The diagnosis of systemic disease from a skin change emphasizes the importance of further investigation of cutaneous vascular lesions.

Urticária de pressão tardia com manifestações sistêmicas: relato de caso / Delayed pressure urticaria with systemic manifestations: case report

A Urticária de Pressão Tardia é considerada uma doença rara, cujo quadro clínico é diferente da urticária clássica e pode envolver manifestações sistêmicas. Sendo assim,o diagnóstico é pouco frequente, até mesmo pelos especialistas. Neste artigo, apresentamos uma paciente, com história típica de lesões desencadeadas por pressão e que, por apresentar febre e leucocitose, foi internada para investigação de quadro infeccioso.

Delayed Pressure Urticaria is considered a rare disease,with clinical diagnosis different from classical urticaria, with possible systemic manifestations. Therefore, it is Frequently underdiagnosed, even by specialists. In this article, the case of a patient with a typical history of pressure-induced lesions is presented. Because the patient had fever and leukocytosis, she was admitted to a hospital for investigation of infection.

Hypocomplementemic Urticarial Vasculitis in Systemic Lupus Erythematosus

Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE.

Morbilidad psiquiátrica y calidad de vida en pacientes con urticaria crónica idiopática / Psychiatric morbidity and quality of life in patients with chronic idiopathic urticaria

Objetivos. Determinar la prevalencia de la morbilidad psiquiátrica en los pacientes con urticaria crónica idiopática (UCI); determinar la calidad de vida relacionada con la salud (HRQL) en los pacientes con UCI en comparación con controles sanos. Métodos. Se seleccionaron 350 pacientes con urticaria crónica, entre el 1/01/2005 y 31/04/2005 en el Departamento de Alergia de la Universidad de Estambul, que cumplieran con los criterios de inclusión: pacientes con diagnóstico de UCI, con edad de entre 18 y 65 años, sin síntomas psicóticos ni retardo mental, con capacidad cognitiva normal y ausencia de enfermedad comórbida. Como control se eligió un grupo de individuos sanos con características demográficas comunes. Todos los participantes recibieron información sobre el estudio mediante consentimiento informado. Se realizó el diagnóstico de UCI descartando, para ello, todas las causas posibles (drogas, químicos, alimentos) mediante dietas de eliminación y con prick tests negativos. Además, se realizaron exámenes de laboratorio: hematología, enzimas hepáticas, hormonas tiroideas, anticuerpos antinucleares y test del suero autólogo. Se utilizó una escala visual analógica, además de entrevistas clínicas para medir la severidad de la enfermedad. Por otra parte, la evaluación psiquiátrica consistió en: entrevistas de forma semiestructurada en donde los participantes completaron un cuestionario de HRQL (resultado de la encuesta de salud) de 36 ítems de forma corta (SF-36) y además una entrevista clínica estructurada por el eje DSM-IV para diagnóstico de enfermedad psiquiátrica en los pacientes con UCI. Resultados. En este estudio se incluyó a 84 pacientes con UCI y 75 controles sanos. La media de edad ± desvío estándar fue 36,83 ± 10,26. El 84% de los pacientes eran mujeres; la duración media (±DE) de la enfermedad fue 6,34 ± 7,2 años y los síntomas fueron intermitentes en el 51% de los pacientes.

Avaliação da associação entre doença auto-imune de tireóide e urticária crônica idiopática / Assessment of association between autoimmune thyroid disease and chronic urticaria

Vários estudos encontraram maior prevalência de Doença Auto-imune de Tireóide (DAT) em pacientes com Urticária Crônica (UC). Essa relação pode ocorrer devido à possível etiologia auto-imune em até um terço dos casos de Urticária Crônica Idiopática (UCI). No entanto, a freqüência de DAT variou de 1,14 por cento a 28,6 por cento. O princípio deste estudo foi determinar se ocorre associação entre DAT e UCI em uma população atendida em um mesmo centro de saúde. Comparamos a freqüência de anticorpos anti-tireoidianos e disfunção tireoidiana entre 49 pacientes com UCI (grupo 1) e 112 controles (grupo 2). Com a finalidade de fortalecer o resultado encontrado, estudamos a prevalência de UCI em 60 pacientes com DAT (grupo 3) comparados com 29 com doença não auto-imune de tireóide (DNAT) (grupo 4). Não encontramos diferença estatística quanto à presença de anticorpos anti-tireoidianos ou disfunção tireoidiana entre os grupos 1 e 2 (12,24 por cento x 9,82 por cento e 12,24 por cento x 7,14 por cento, respectivamente). O mesmo ocorreu quanto à presença de UCI entre os grupos 3 e 4 (3,33 por cento x 3,44 por cento). Em nosso estudo não foi possível demonstrar uma relação entre DAT e UCI, o que significa que diferentes populações podem apresentar maior ou menor grau de associação entre essas doenças.

Several studies found a higher prevalence of Autoimmune Thyroid Disease (ATD) in patients with Chronic Urticaria (CU). This relationship may be due to the possible autoimmune etiology in up to one third of the cases of Chronic Idiopathic Urticaria (CIU). However, the frequency of ATD ranged from 1.14 percent to 28.6 percent. The study began by determining whether there is an association between ATD and CU, in a population seen at the same clinic. We compared the frequency of anti-thyroid antibodies and thyroid dysfunction in 49 patients with CIU (group 1) and 112 controls (group 2). In order to support the result found, we studied the prevalence of CIU in 60 patients with ATD (group 3) and compared with 29 patients who had non-immune thyroid disease (NITD) (group 4). We did not find a statistical difference for the presence of anti-thyroid antibodies or thyroid dysfunction between groups 1 and 2 (12.24 percent x 9.82 percent and 12.24 percent x 7.14 percent, respectively). The same occurred for the presence of CIU among groups 3 and 4 (3.33 percent x 3.44 percent). In our study it was not possible to demonstrate a relationship between ATD and CIU, which means that different populations may present a higher or lower degree of association between these illnesses.

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.