Guía clínica chilena de urticaria crónica espontánea / Chilean guidelines for chronic urticaria

Chronic urticaria (CU) is characterized by itchy wheals, angioedema or both lasting six weeks or more. It is classified as spontaneous (CSU) and inducible urticaria (CIndU), depending whether there is an identifiable trigger or not. CSU is the predominant form, affecting more than 75% of patients, although overlaps often occur. The prevalence of CSU throughout life is around 1.8% and predominates in women at a ratio of 2:1. The higher incidence of CSU is between 20 and 40 years of age and lasts between one to five years. However, in up to 20% of patients the disease may last longer. CSU not only hampers quality of life, but also affect performance at work and school. The diagnosis of CSU is mainly clinical, and laboratory tests are required depending on the clinical history of the patient. Extensive laboratory tests are usually unnecessary. Second generation anti-histamines are used as first line treatment for CSU treatment. In refractory patients, systemic treatments, such as cyclosporine or omalizumab are suggested. We herein report the first Chilean CSU guidelines.

Deferasirox-induced urticarial vasculitis in a patient with myelodysplastic syndrome

Abstract Deferasirox is an iron chelator agent used in the treatment of diseases with iron overload, such as thalassemia and myelodysplastic syndrome. Although the majority of adverse reactions of deferasirox involve gastrointestinal symptoms and increase in serum creatinine and transaminases, skin rashes, such as maculopapular and urticarial eruptions, have also been reported. This study reports a case of myelodysplastic syndrome with urticarial vasculitis due to deferasirox therapy. Drug eruption was been confirmed by means of a challenge test, together with histopathological and clinical findings. To the best of our knowledge, we report the first case of deferasirox-induced urticarial vasculitis. Physicians should be aware of the possibility of urticarial vasculitis on deferasirox therapy and the fact that the discontinuation of the drug generally results in improvement.

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.

Follicular traction urticaria

Abstract Inducible urticaria is a heterogeneous subgroup of chronic urticarias caused by a wide variety of environmental stimuli, such as exercise, cold, heat, pressure, sunlight, vibration, and water. A new term, follicular traction urticaria, was suggested as an unusual form of inducible urticarias. We report a patient who was diagnosed with follicular traction urticaria.

Chronic urticaria in adults: state-of-the-art in the new millennium

Chronic urticaria has been explored in several investigative aspects in the new millennium, either as to its pathogenesis, its stand as an autoimmune or auto-reactive disease, the correlation with HLA-linked genetic factors, especially with class II or its interrelation with the coagulation and fibrinolysis systems. New second-generation antihistamines, which act as good symptomatic drugs, emerged and were commercialized over the last decade. Old and new drugs that may interfere with the pathophysiology of the disease, such as cyclosporine and omalizumab have been developed and used as treatments. The purpose of this article is to describe the current state of knowledge on aspects of chronic urticaria such as, pathophysiology, diagnosis and the current therapeutic approach proposed in the literature.

Cutaneous lesions and finger clubbing uncovering hypocomplementemic urticarial vasculitis and hepatitis C with mixed cryoglobulinemia / Lesoes cutaneas e baqueteamento digital reveladores de vasculite urticariforme hipocomplementemica e hepatite C com crioglobulinemia mista

Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia. The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. The development of hypocomplementemic urticarial vasculitis in the context of chronic hepatitis C is exceedingly rare and possible pathogenic mechanisms are discussed.

A vasculite urticariforme é uma entidade clinico-patológica rara caracterizada por lesões urticariformes com duração superior a 24 horas e uma vasculite leucocitoclásica na histologia. É dividida em normo e hipocomplementêmica. Os autores relatam o caso de uma mulher saudável de 49 anos, com lesões cutâneas intensamente pruriginosas e baqueteamento digital com 1 ano de evolução. O estudo efectuado permitiu efectuar os diagnósticos de vasculite urticariforme hipocomplementêmica, hepatite C crônica e crioglobulinêmia mista tipo II. A doente iniciou tratamento sintomático e foi referenciada para a Gastroenterologia para orientação da hepatite, com melhoria progressiva das lesões cutâneas. O desenvolvimento de vasculite urticariforme hipocomplementêmica no contexto de hepatite C crónica é raro e os possíveis mecanismos patogênicos são discutidos.

Urticaria crónica / Chronic urticaria

La urticaria crónica (VC), es una variante común de la urticaria que se define como un síndrome reactivo de la piel y mucosas caracterizado por la presencia de placas eritematosas, habones o ronchas edematosas y pruriginosas que se prolonga durante más de seis semanas. La etiología de la VC no es muy clara en la mayoría de los casos, multitud de causas se han implicado. La patogenia es compleja. El conocimiento y estudio de cada uno de los factores celulares y solubles, su interacción dinámica, vías efectoras y trama de citoquinas conducirá al logro de una terapéutica efectiva y racional de la urticaria crónica.

Chronic urticaria (VC) is a common variant of urticaria which is defined as a reactive skin and mucous membranes syndrome characterized by the presence of plaques erythematous, wheals, or swollen and itchy hives that lasts more than six weeks. The etiology of the VC is not very clear in the majority of cases, multitude of causes have been implicated. The pathogenesis is complex. Knowledge and study of each cell and soluble factors, their dynamic interaction, effector pathways and cytokines plot will lead to the achievement of an effective and rational therapy of chronic urticaria.

Sensibilización al maní en pacientes venezolanos con enfermedades atópicas y/o urticaria / Sensitization to peanut in Venezuelan patients with atopic diseases and / or uticaria

La sensibilización y las manifestaciones alérgicas al maní se han incrementado últimamente a nivel mundial, constituyendo el mismo la causa principal de anafilaxia por alimentos. Como la prevalencia de alergia al maní varía de acuerdo a las regiones nos propusimos evaluar, en una etapa preliminar, la sensibilización al maní por pruebas cutáneas (“skin prick test”) en pacientes venezolanos atópicos y/o con urticarias que acudieron a la Consulta ambulatoria de Alergía del Instituto de Inmunología. El 5,4 % de los pacientes manifestó algún tipo de manifestación cutánea o respiratoria al ingerir maní. Se demostró sensibilización al maní por pruebas cutánea en el 6,5 % de los pacientes. Sin embargo, un porcentaje pequeño (2 %) de ellos mostró, en conjunto, pruebas cutáneas positivas y síntomas a la ingesta del maní. Ningún paciente refirió síntomas severos tras la ingestión de maní. La mayoría de los pacientes con pruebas positivas al maní, también mostraron pruebas positivas a otros alimentos. Estos resultados concuerdan con la percepción de los médicos venezolanos de una baja frecuencia de reacciones adversas, especialmente graves, a la ingesta de maní en nuestro país

Peanut allergy and sensitization incidence has increased world wide to become the first cause of food anaphylaxis. Since the prevalence of peanut allergy changes according to geographical areas, the aim of the study was to assess, in a preliminary report, peanut allergy incidence by skin prick test in atopic Venezuelan patients with atopy and or urticaria from the outpatient allergy clinic of the Institute of Immunology. Cutaneous or respiratory manifestations after peanut ingestion was observed in 5.4 % of the patients studied. Cutaneous test was positive in 6.5 % of patients. In the other hand, a small group (2 %), showed positive skin test along with symptoms after peanut ingestion. None of the patients had severe reactions. Most of the patients with peanut positive skin test were positive to other food allergens. These results are in accordance with the general clinical perception of small frequency of adverse reaction, specially the most serious ones, to peanut ingestion in our country

Immunoelectron microscopy study of superficial skin nerves in drug-induced acute urticaria / Estudo de microscopia imunoeletrônica dos nervos superficiais da pele na urticária aguda induzida por medicamentos

BACKGROUND: Few studies have evaluated the ultrastructure of the superficial skin nerves in urticaria. OBJECTIVE: The objective of this study was to describe findings in superficial skin nerves in cases of drug-induced acute urticaria. METHODS: Seven patients with drug-induced acute urticaria were included in the study. Skin biopsies were obtained from the urticarial lesion and from the apparently normal skin. The 14 fragments collected were processed for immunogold electron microscopy using single stains for antitryptase and anti-FXIIIa antibodies, as well as double immunogold labeling for both. RESULTS: Some sections showed mast cells in the process of degranulation. Following double immunogold staining, 10 nm (FXIIIa) and 15 nm (Tryptase) gold particles were found together throughout the granules in mast cells, indicating that tryptase and FXIIIa are located inside each one of the granules of these cells. Interestingly, we found strong evidence of the presence of tryptase and factor XIIIa in the superficial skin nerves of these patients, both in cases of urticarial lesions (wheals) and in the apparently normal skin. CONCLUSIONS: Tryptase and FXIIIa are present in the superficial nerves of the skin in drug-induced acute urticaria. This is the first report of tryptase and FXIIIa expression in the superficial skin nerves of patients with urticaria. Tryptase may be participating in neural activation in these patients, while FXIIIa may be present in the nerves to guarantee the functional integrity of structures.

FUNDAMENTOS: Poucos autores têm estudado a ultraestrutura dos nervos superficiais na urticária. OBJETIVO: Descrever os achados nos nervos cutâneos superficiais em casos de urticária aguda induzida por medicamentos. MÉTODOS: Sete pacientes com urticária aguda induzida por medicamentos foram incluídos no estudo. Foram obtidas biopsias da pele da lesão urticariforme e da pele aparentemente normal. Os 14 fragmentos coletados foram processados usando imunomarcação com ouro para anticorpos anti-triptase e anti-FXIIIa separadamente, além da dupla imunomarcação com ambos anticorpos. A seguir as amostras foram submetidas à análise por microscopia imunoeletrônica. RESULTADOS: Alguns cortes demonstraram mastócitos em processo de degranulação. Após a imunomarcação dupla, partículas de ouro de 10 nm (FXIIIa) e partículas de ouro de 15 nm (Triptase) apresentavam-se juntas em grânulos de mastócitos indicando que a triptase e o FXIIIa se localizam dentro de cada um dos grânulos dessas células. Curiosamente, foi encontrada uma forte evidência da presença da triptase e do fator XIIIa nos nervos superficiais dos pacientes avaliados, tanto em lesões urticadas, como na pele aparentemente normal. CONCLUSÕES: A triptase e o FXIIIa estão presentes nos nervos superficiais da pele na urticária aguda medicamentosa. Este é o primeiro relato da expressão de triptase e de FXIIIa nos nervos superficiais na urticária. A triptase poderia estar participando da ativação neural nos pacientes estudados. O FXIIIa poderia estar presente nos nervos, com a finalidade de manter a integridade funcional dessas estruturas.

Urticaria aguda / Acute urticaria

La urticaria es una reacción de la piel caracterizada clínicamente por la aparición de elevaciones cutáneas eritematosas o blanquecinas, edematosas, pruriginosas y transitorias, que pueden localizarse en cualquier parte de la superficie cutáneao en las mucosas. Su etiología es variada y sus mecanismos etiopatogénicos también suelen ser múltiples. La lesión típica es el habón o roncha, el cuál cambia de lugar en horas, su forma y tamaño son variables, puede persistir minutos, horas o semanas. La mayoría de las urticarias no presenta sintomatología sistémica. Suele clasificarse regularmente en urticaria aguda, cuando lleva menos de 6 semanas de aparición, y en urticaria crónica, cuando lleva más de 6 semanas. El diagnóstico es fundamentalmente clínico. Es una entidad frecuente que por su gran expresividad motivan constantes y reiteradas consultas a los servicios de urgencia. En este artículo se examinarán los mecanismos que llevan a la urticaria y angioedema, y se revisará la patogénesis, los aspectos clínicos, los estudios de laboratorio necesarios y el tratamiento de la urticaria aguda.

The urticaria is a skin reaction characterized clinically by the on set of erythematous skin elevations or whitish, oedematous,pruritic and transient, you can locate anywhere on the surface of the skin or the mucous membranes. Its etiology is varied and its mechanisms etiopathogenic also tend to be multiple. The typical lesion is the wheal or bump, which changes from place with inhours, its size and shape are variable, may persist for minutes, hours or weeks. The majority of the hives does not pose systemic symptoms. Usually qualify regularly in acute urticaria, when it takes less than 6 weeks of appearance, and chronic urticaria, when it takes over 6 weeks. The diagnosis is primarily clinical. Itis a common entity that by their expressivity motivate constants and repeated consultations to emergency services. This article will xamine the mechanisms that lead to angioedema and urticaria, he pathogenesis, clinical aspects, necessary laboratory studies and eatment.

Dermatoses neutrofílicas: parte I / Neutrophilic dermatoses: part I

Os autores apresentam uma revisão das dermatoses neutrofílicas que possuem grande repercussão à saúde dos pacientes: síndrome de Sweet, pioderma gangrenoso, doença de Behçet e urticária neutrofílica. São discutidos, baseados nos resultados e conclusões de estudos relevantes publicados recentemente e na experiência dos autores, os principais aspectos clínicos, as importantes alterações histopatológicas e as opções para o manejo.

The authors present a review of neutrophilic dermatoses that have great impact on the health of patients: Sweet syndrome, pyoderma gangrenosum, Behçet's disease and neutrophilic urticaria. Major clinical aspects, histopathological changes and management options are discussed based on the results and conclusions of relevant studies recently published and on the authors' experience.

Pemphigoid gestationis: clinical and laboratory evaluation

INTRODUCTION: Pemphigoid gestationis, also known as herpes gestationis, is a rare autoimmune blistering disease associated with pregnancy. It usually occurs during the second or third trimester, but it may be present at any stage of pregnancy or the puerperium. The clinical, histologic, and immunopathological features of pemphigoid gestationis are similar to those of the pemphigoid group of disorders. METHODS: We hereby report seven patients who were diagnosed with pemphigoid gestationis and followed at the Autoimmune Blistering Disease Clinic in the Department of Dermatology of the University of Sao Paulo Medical School between 1996 and 2008. DISCUSSION: Demographic and clinical findings, such as median age, sites of involvement, and gestational age of onset of our patients, coincide with those described in previous reports. The majority of patients (85 percent) exhibited complement C3 or C3 and immunoglobulin G (IgG) deposition along the basement membrane zone (BMZ) on immunofluorescence. Herpes gestationis factor (HG) factor was postitive in four out of six patients (67 percent), and three out of five patients recognized the bullous pemphigoid recombinant antigen (BP180) by ELISA. CONCLUSION: This study revealed a good outcome of the newborns from pemphigoid gestationis affected mothers, based on the absence of pemphigoid gestationis cutaneous lesions, mean birth weight, and normal Apgar scores and gestational age at birth.

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

Popular urticaria among atopic and Non - atopic pediatric patients in Suez canal region: a parasitological and immunological study

This study aimed to identify the species of insects that might be responsible for papular urticaria [PU] and to study some immunological aspects of the disease among affected atopic patients [AP] and non- atopic patients [NAP]. This study included 40 urban pediatric patients with PU of different duration and ten healthy age matched control subjects. It was concluded that during summer, Culex pipiens is the most common cause of PU in urban areas of Suez-Canal region, followed by human fleas. Antibodies of the IgE class play a central role in insect bite allergy and PU, while antibodies of IgG class may be formed to either sensitize mast cells and basophils for a short period or help natural desensitization during a long-term exposure. Helper T-lymphocytes may initiate the immunologic response of PU, while suppressor T-lymphocytes may serve to terminate it