Urticaria, una forma de presentación atípica de COVID-19. Reporte de caso / Urticaria, an atypical presentation of COVID-19. Case report

Desde su aparición en Wuhan, China, y luego de más de dos años de ser declarada como pandemia, la COVID-19 ha provocado más de cinco millones de muertes en el mundo. Es ampliamente conocido que no solo afecta al sistema respiratorio, sino que aparecen manifestaciones digestivas, cardiovasculares, endocrinometabólicas, neurológicas, renales y cutáneas. El espectro dermatológico que guarda relación con la COVID-19 se ha definido en cinco grupos principales de manifestaciones: lesiones maculopapulares, lesiones acrales, patrón urticariforme, patrón vesiculoso y lesiones de livedo o necrosis, según su frecuencia de aparición. Se describe un caso con presencia de rash urticariforme como único síntoma reportado en un paciente con diagnóstico de COVID-19(AU)

Since its appearance in Wuhan, China, and after more than two years after being declared a pandemic, COVID-19 has caused more than five million deaths in the world. It is widely known that it not only affects the respiratory system, but also has digestive, cardiovascular, endocrine-metabolic, neurological, renal and skin manifestations. The dermatological spectrum that is related to COVID-19 has been defined in five main groups of manifestations: maculopapular lesions, acral lesions, urticarial pattern, vesicular pattern and livedoid or necrotic lesions, according to their frequency of appearance. A case is described with the presence of urticarial rash as the only symptom reported in a patient diagnosed with COVID-19(AU)

Analysis of distribution characteristics of specific immunoglobulin E in 8 092 children with eczema and urticaria in a hospital of pediatric in Tianjin City / 中华预防医学杂志

To investigate the common specific immunoglobulin E(sIgE) in children with eczema and urticaria, compare the allergies in children with different diseases, genders and ages, and provide the scientific basis for the prevention, diagnosis and treatment. A retrospective study was conducted to analyze the children who were suspected of eczema and urticaria and tested for serum sIgE in the Tianjin Children's Hospital from December 2019 to August 2021. A total of 8 092 serum samples were tested for ten food allergens and ten inhaled allergens. The method was the enzyme-linked immune capture assay. The allergen epidemiological characteristics were statistically analyzed by Chi square test based on the children's characteristics and factors such as different sexes and ages and by the mass data. The results showed that the positive rate of eczema was 64.42%(5 213/8 092), and the urticaria was 35.58%(2 879/8 092). The positive rate of specific IgE was 66.65%(5 393/8 092), the food allergens was 61.74%(4 996/8 092), and the inhaled allergens was 34.85%(2 820/8 092). The top three positive rates of food allergens were egg 46.65%(3 775/8 092), milk 32.64%(2 641/8 092) and wheat flour 15.08%(1 220/8 092). The top three positive rates of inhaled allergens were house dust 21.40%(1 732/8 092), Alternaria 11.78%(953/8 092) and Dermatophagoides farinae 7.33%(593/8 092). The positivity of food allergens and inhaled allergens was significantly different in different age groups. The positive rates of food allergens in different age groups were 48.92%(947/1 936) in<1 year old, 72.28%(2 680/3 708) in 1-3 years old, 64.58%(919/1 423) in 4-6 years old and 43.90%(450/1 025) in>6 years old. The positive rates of inhaled allergens in different age groups were 17.67%(342/1 936) in<1 year old, 36.35%(1 348/3 708) in 1-3 years old, 46.38%(660/1 423) in 4-6 years old and 45.85%(470/1 025) in>6 years old. The top six positive rates of allergens of eczema were the same with urticaria, which were egg, milk, house dust, wheat flour, Alternaria and Dermatophagoides farinae. The allergens (greater than or equal to grade 4) differed in children with eczema and urticaria. Moreover, there were significant differences in the positive rates of Alternaria, egg, wheat flour, crab and shrimp. In conclusion, this study can reflect the epidemic characteristics of allergens in children with eczema and urticaria to a certain extent. There were significant differences in the positive rates of allergens between different age groups. It is necessary to reasonably avoid the high-risk allergens according to the epidemiological characteristics and clinical symptoms, which provide valuable information for the prevention, diagnosis and treatment of allergic diseases.

Analysis of distribution characteristics of specific immunoglobulin E in 8 092 children with eczema and urticaria in a hospital of pediatric in Tianjin City / 中华预防医学杂志

To investigate the common specific immunoglobulin E(sIgE) in children with eczema and urticaria, compare the allergies in children with different diseases, genders and ages, and provide the scientific basis for the prevention, diagnosis and treatment. A retrospective study was conducted to analyze the children who were suspected of eczema and urticaria and tested for serum sIgE in the Tianjin Children's Hospital from December 2019 to August 2021. A total of 8 092 serum samples were tested for ten food allergens and ten inhaled allergens. The method was the enzyme-linked immune capture assay. The allergen epidemiological characteristics were statistically analyzed by Chi square test based on the children's characteristics and factors such as different sexes and ages and by the mass data. The results showed that the positive rate of eczema was 64.42%(5 213/8 092), and the urticaria was 35.58%(2 879/8 092). The positive rate of specific IgE was 66.65%(5 393/8 092), the food allergens was 61.74%(4 996/8 092), and the inhaled allergens was 34.85%(2 820/8 092). The top three positive rates of food allergens were egg 46.65%(3 775/8 092), milk 32.64%(2 641/8 092) and wheat flour 15.08%(1 220/8 092). The top three positive rates of inhaled allergens were house dust 21.40%(1 732/8 092), Alternaria 11.78%(953/8 092) and Dermatophagoides farinae 7.33%(593/8 092). The positivity of food allergens and inhaled allergens was significantly different in different age groups. The positive rates of food allergens in different age groups were 48.92%(947/1 936) in<1 year old, 72.28%(2 680/3 708) in 1-3 years old, 64.58%(919/1 423) in 4-6 years old and 43.90%(450/1 025) in>6 years old. The positive rates of inhaled allergens in different age groups were 17.67%(342/1 936) in<1 year old, 36.35%(1 348/3 708) in 1-3 years old, 46.38%(660/1 423) in 4-6 years old and 45.85%(470/1 025) in>6 years old. The top six positive rates of allergens of eczema were the same with urticaria, which were egg, milk, house dust, wheat flour, Alternaria and Dermatophagoides farinae. The allergens (greater than or equal to grade 4) differed in children with eczema and urticaria. Moreover, there were significant differences in the positive rates of Alternaria, egg, wheat flour, crab and shrimp. In conclusion, this study can reflect the epidemic characteristics of allergens in children with eczema and urticaria to a certain extent. There were significant differences in the positive rates of allergens between different age groups. It is necessary to reasonably avoid the high-risk allergens according to the epidemiological characteristics and clinical symptoms, which provide valuable information for the prevention, diagnosis and treatment of allergic diseases.

Guia prático de urticária para grupos especiais de pacientes / Practical guide to urticaria for special patient groups

A urticária crônica é uma condição que afeta mais de um milhão de brasileiros, com grande impacto na qualidade de vida. Mesmo com diretrizes bem difundidas para o seu diagnóstico e tratamento, seu manejo pode ser desafiador em pacientes pediátricos, idosos e gestantes. Para auxiliar o médico especialista nestes casos, o Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia elaborou esta revisão com as principais dúvidas e dificuldades referentes ao tema nestes grupos de pacientes.

Chronic urticaria is a condition that affects more than a million Brazilians with a significant impact on quality of life. Although there are well-established guidelines for diagnosis and treatment, the management of chronic urticaria may be challenging in pediatric, older, and pregnant patients. With the purpose of helping specialists manage these cases, the Urticaria Scientific Department of the Brazilian Association of Allergy and Immunology prepared this review with the most common doubts and difficulties about this topic in those patient groups.

Guia prático de urticária aguda / Practical guide to acute urticaria

A urticária aguda é uma causa frequente de consulta com alergistas, caracterizada por urticas e/ou angioedema. Embora autolimitada e benigna, pode causar desconforto significativo e raramente representar uma doença sistêmica grave ou reação alérgica com risco de vida. Nesta revisão, elaborada pelo Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia, foram abordadas as principais questões referentes ao tema para auxiliar o médico especialista e generalista.

Acute urticaria is a frequent cause of consultations with allergists, being characterized by wheals and/or angioedema. Although self-limited and benign, it may cause significant discomfort and uncommonly represent a serious systemic disease or life-threatening allergic reaction. In this review prepared by the Urticaria Scientific Department of the Brazilian Association of Allergy and Immunology, the main questions about this topic are addressed to help specialists and general practitioners.

Leguminosas como causa infrecuente de alergia alimentaria en Colombia: reporte de dos casos y revisión de la literatura / Legumes as an infrequent cause of food allergy in Colombia: report of two cases and review of the literature

La alergia alimentaria se ha venido incrementando a nivel mundial, afectando alrededor del 1,5 % a 2,5 % de los adultos y 6 % de los niños, y tiene un gran impacto en la calidad de vida de los pacientes y sus cuidadores, debido a las dietas de restricción. Los alérgenos más prevalentes son la leche, el huevo, el trigo, la soja, los frutos secos, el maní, el pescado y los mariscos. Las leguminosas mejor estudiadas son el maní y la soja; otras leguminosas como las lentejas, garbanzos y arvejas representan la quinta causa de alergia alimentaria en el área mediterránea, en Turquía y en la India, siendo menos prevalentes en otras áreas geográficas. La alergia a las leguminosas es una entidad infrecuente en Colombia, se desconoce la prevalencia en el país. Describimos los primeros dos casos de anafilaxia por lentejas reportados en el país. Ambos pacientes menores de 18 años, con reacciones adversas tras la ingesta de leguminosas, en las cuales se demuestra alergia mediada por IgE a las lentejas y además sensibilización en el primer caso a las arvejas y garbanzos, y en el segundo caso a los frijoles. Diferentes datos sobre la prevalencia se han descrito en varias áreas geográficas, siendo mayor en países con dietas mediterráneas. Las reacciones mediadas por IgE suelen aparecer incluso con el alimento altamente cocido, debido a la termo-estabilidad de las proteínas. La reactividad cruzada más frecuente se relaciona con los garbanzos y las arvejas

Food allergy has been increasing worldwide. Affects around 1.5% to 2.5% of adults and 6% of children, and has a great impact on the quality of life of patients and their caregivers, due to restricted diets. The most prevalent allergens are milk, egg, wheat, soy, tree nuts, peanuts, fish and shellfish. The best studied legumes are peanuts and soybeans; other legumes such as lentils, chickpeas and peas represent the fifth cause of food allergy in the Mediterranean area, Turkey and India, being less prevalent in other geographical areas. Allergy to legumes is not common in Colombia, the prevalence in the country is unknown. We describe the first two cases of legumes anaphylaxis reported in the country. Both patients were under 18 years of age, with adverse reactions after ingesting legumes, in which IgE-mediated allergy was demonstrated; in the first case to lentils, peas and chickpeas, and in the second case, to lentils and beans. Different data on prevalence have been described in various geographical areas, being higher in countries with Mediterranean diets. IgE-mediated reactions usually appear even with highly cooked food, due to the thermo-stability of proteins. The most frequent cross-reactivity is related to chickpeas and peas

Pitiriasis rosada como manifestación dermatológica de infección por SARS-CoV-2: a propósito de un caso / Pityriasis rosea as a cutaneous manifestation associated with SARS-CoV-2 infection: case report

Las manifestaciones cutáneas relacionadas a la infección por el coronavirus SARS-CoV-2, causante de COVID-19, se han descrito entre el 0,2% y 20,4% de las personas que cursan con esta enfermedad. Las más frecuentemente descritas son: lesiones maculopapulares (47%), lesiones acrales eritematosas con vesículas o pústulas (pseudoperniosis) (19%), urticariales (19%), lesiones vesiculosas (9%) y livedo/necrosis (6%). En particular, la pitiriasis rosada es una dermatosis autolimitada de etiología desconocida, sin embargo, se ha visto asociada a la infección por SARS-CoV-2, con algunos reportes de casos en la literatura. El mecanismo fisiopatológico de las lesiones cutáneas en COVID-19 no es claro, y se han planteado algunas teorías, entre las cuales está el papel que juega la enzima convertidora de angiotensina 2 (ACE2) utilizada por el virus para infectar las células, los infiltrados linfocíticos, los depósitos de factores del complemento en la piel, y la reactivación de virus latentes como los herpes virus humanos. Se presenta el caso de una paciente con pitiriasis rosada asociada a COVID-19 y se describen los casos reportados hasta la fecha

O estado da arte das síndromes autoinflamatórias associadas à criopirina / The state of the art of cryopyrin-associated periodic syndromes

As síndromes autoinflamatórias associadas à criopirina (CAPS) compreendem um grupo espectral de doenças raras autoinflamatórias. Todas estas doenças estão relacionadas ao inflamassoma NLRP3, sendo que de 50-60% dos pacientes apresentam mutações ao longo do gene NLRP3. Clinicamente, febre recorrente associada à urticária neutrofílica e outros sintomas sistêmicos são o grande marco clínico, comum a todo o espectro. O bloqueio da interleucina-1 trouxe grande alívio ao tratamento destas desordens, mas variações na resposta clínica podem ser observadas, principalmente nos espectros mais graves. Neste trabalho os autores trazem uma revisão do estado da arte das doenças autoinflamatórias CAPS. Foi realizado levantamento de literatura e, ao final, 49 artigos restaram como base para construção do texto final. O trabalho traz de forma narrativa os principais pontos relacionados a imunofisiopatologia, manifestação clínica, diagnóstico, tratamento, complicações e novas armas diagnósticas, e terapia gênica.

Cryopyrin-associated periodic syndromes (CAPS) comprise a spectrum of rare autoinflammatory disorders. They are all related to the NLRP3 inflammasome, and 50-60% of the patients harbor mutations along the NLRP3 gene. Clinically, recurrent fever associated with neutrophilic urticaria and other systemic symptoms are a hallmark of all the disorders in the spectrum. Biologic drugs that can block interleukin-1 were a milestone for the treatment of such rare diseases, although variability in clinical response to this therapeutic intervention were observed, especially in those affected by severe phenotypes. In this paper, the authors provide a state-of-the-art review of CAPS. A literature search was performed and, finally, 49 articles remained for the construction of the final manuscript. The article presents a narrative review focused on the topics related to immune pathophysiology, clinical manifestations, diagnosis, treatment, complications and new therapeutic options, and gene therapy.

Anafilaxia ao ácido poli-L-láctico / Poly-L-lactic acid anaphylaxis

Paciente feminina, 47 anos, previamente hígida, apresentou reação anafilática associada ao uso do ácido poli-L-láctico (PLLA). Imediatamente após a administração do bioestimulador, a paciente referiu edema de face que evoluiu para urticária generalizada, edema em membros inferiores e tremores. Posteriormente, apresentou edema de língua e dificuldade para falar. Teste de puntura com extratos de PLLA na concentração pura 1:1 e testes intradérmicos na diluição 1:10 e 1:100 mostrou-se positivo. Paciente negou cofatores no dia do procedimento e alergias prévias. O presente artigo descreve o primeiro caso da literatura de anafilaxia ao PLLA, onde se discute aspectos da reação anafilática e exames usados para o diagnóstico.

A previously healthy 47-year-old woman had an anaphylactic reaction caused by poly-L-lactic acid (PLLA). Immediately after the administration of the biostimulating agent, the patient reported facial edema, which progressed to generalized urticaria, lower extremity edema, and tremors. Then she had tongue edema and difficulties to talk. A prick test with pure PLLA extracts (1:1) and intradermic tests (1:10 and 1:100 dilutions) were positive. The patient denied cofactors on procedure day as well as previous allergies. This is the first case report of anaphylaxis due to PLLA and includes a discussion of aspects of the anaphylactic reaction and tests that were used to provide the diagnosis.

What lessons can we learn? Clinical and epidemiological retrospective analysis of 267 patients with urticaria in a Brazilian tertiary center

Abstract Background: There are few epidemiological studies of urticaria, published in the indexed literature (PubMed/Medline). Objective: The study aimed to evaluate the epidemiological and clinical data among patients with urticaria/angioedema attending a reference clinic in Brazil. Methods: Two hundred sixty-seven patients were evaluated retrospectively considering demographic data, time course of the disease, triggering symptoms, the presence of angioedema, complementary laboratory tests including total blood count, reactive-C protein, erythrocyte sedimentation rate, IgE serum levels, and other, as necessary. Results: The most commonly diagnosed type of urticaria was chronic spontaneous urticaria (56.93%). Angioedema was associated with chronic urticaria in 108 patients (40.08%). Study limitations: Unicentered and retrospective. Conclusion: Some relevant findings in this study are the observation of a female prevalence of cases (4-females: 1-man), a result more elevated than demonstrated in previous studies in Europe and Asia, the median age was 43-years old and the delay of time between the diagnosis of urticaria and the admission for treatment in a specialized center was approximately 2-years. Other multicenter studies can better establish these differences in Brazilian patients.

Vacina COVID-19 em pacientes com urticária ­ Posicionamento do Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia (ASBAI) / COVID-19 vaccine in patients with urticaria ­ Position statement of the Scientific Department of Urticaria of the Brazilian Association of Allergy and Immunology (ASBAI)

Com o início do programa de vacinação contra a COVID-19 no Brasil, surgiu uma série de questionamentos relacionados ao uso dos imunizantes em pacientes com doenças imunoalérgicas. Neste documento, o Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia (ASBAI) se posiciona revisando as principais dúvidas relacionadas à imunização para COVID-19 em pacientes com urticária.

As the COVID-19 vaccination program started in Brazil, many questions have arisen regarding the use of vaccines in patients with immune-allergic diseases. In this document, the Scientific Department of Urticaria of the Brazilian Association of Allergy and Immunology takes a stand by reviewing the main queries regarding COVID-19 immunization in patients with urticaria.

Urticária e a COVID-19 / Urticaria and COVID-19

A pandemia de COVID-19 afetou drasticamente a vida de todos ao redor do planeta, interferindo também na forma de atuarmos como médicos e especialistas. Neste artigo revisamos aspectos importantes da infecção pelo novo coronavírus e sua relação com a urticária.

The COVID-19 pandemic has dramatically affected people's lives around the world and has interfered with how we act as physicians and specialists. In this paper, we review important aspects of the new coronavirus infection and its connection with urticaria.

Identification of a thaumatin-like protein as a new allergen in persimmon (Diospyros kaki) with cross-reactivity with banana (Musa acuminata) / Identificação de uma proteína semelhante à taumatina como um novo alérgeno no caqui (Diospyros kaki) com reatividade cruzada com a banana (Musa acuminata)

Allergy to persimmon (Diospyros kaki ) has been only rarely reported. The antigenic composition of the fruit is not entirely known. Thaumatin-like proteins (TLPs) have been described as allergens in pollens and various fruits, such as kiwi and banana, but not in persimmon. We report the case of a 22-year-old man, with persistent moderate-to-severe allergic rhinitis, sensitized to house dust mites. The patient describes an episode of oral mucosa and ear canal pruritus, followed by diffuse urticaria, which rapidly evolved to dysphonia, dyspnea, and dizziness, after eating raw persimmon. A few months later he developed similar cutaneous symptoms accompanied by nausea, vomiting, abdominal colic, and hypotension immediately after the intake of banana. The prick-prick test with raw persimmon and banana were positive, as well as the serum specific IgE to the extract of these fruits. The ImmunoCAP ISAC_112i test demonstrated a positive specific IgE against Act d 2 (kiwi thaumatin), which is homologous to banana TLP (Mus a 4). Serum IgE inhibition test with "sponge" of Diospyros kaki ImmunoCAP (f301) showed partial inhibition (40%) of IgE to Act d 2. This raises the suspicion that a TLP is at least partially responsible for the referred sensitization. This patient is sensitized to Diospyros kaki and Musa acuminata. An anaphylactic reaction to consumed persimmon, presumably as a result from cross-allergy with banana thaumatin was diagnosed in our patient. Thaumatin has not been previously described as an allergen of persimmon with cross-reactivity with banana, and in vitro with Act d 2 (kiwi TLP).

A alergia ao caqui (Diospyros kaki ) tem sido raramente documentada, não sendo a composição antigênica da fruta totalmente conhecida. Proteínas semelhantes à taumatina (TLPs) foram descritas como alergênicos em pólens e várias frutas, como no kiwi e banana, mas não no caqui. Apresenta-se o caso de um doente de 22 anos, com rinite alérgica persistente moderadagrave, sensibilizado a ácaros do pó doméstico. O doente refere episódio de prurido na mucosa oral e canal auditivo, seguido de urticária generalizada, que rapidamente evoluiu para disfonia, dispneia e tontura, após ingestão de caqui. Poucos meses depois, desenvolveu sintomas cutâneos semelhantes, acompanhados de náuseas, vómitos, cólica abdominal e hipotensão imediatamente após ingestão de uma banana. O teste cutâneo por picada com caqui e banana em natureza foram positivos, bem como o doseamento de IgE específica. O teste ImmunoCAP ISAC_112i identificou a presença de IgE específica para Act d 2 (taumatina do kiwi), homóloga da TLP da banana (Mus a 4). O estudo de inibição ImmunoCAP ISAC com "esponja" de Diospyros kaki (f301) produziu uma inibição parcial (40%) da ligação de IgE a Act d 2, permitindo presumir que uma proteína semelhante à taumatina é, pelo menos, parcialmente responsável pela referida sensibilização. Este doente encontra-se sensibilizado a Diospyros kaki e Musa acuminata. Uma anafilaxia ao caqui ingerido, presumivelmente resultante de reatividade cruzada com a taumatina da banana foi diagnosticada. Não estão descritas na literatura TLPs como alergênicos do caqui com reatividade cruzada com a banana e com Act d 2 in vitro (TLP do kiwi).

Evolução da infecção por COVID-19 em um grupo de pacientes com erros inatos da imunidade / Evolution of a Brazilian group of patients with inborn errors of immunity with COVID-19

Introdução: O novo coronavírus causou uma pandemia e desafio na saúde pública em todo o mundo. Até hoje muitos mecanismos do vírus no hospedeiro foram desvendados, cujo conhecimento é essencial para entender a evolução clínica e desenvolver uma estratégia de terapia adequada para a infecção com COVID-19. Contudo, pouco se sabe da infecção por COVID-19 em pacientes com erros inatos da imunidade (EII), principalmente em pacientes com síndromes autoinflamatórias. Objetivo: Descrever a evolução de pacientes com erros inatos da imunidade acometidos por SARS-CoV-2 em um centro de referência em doenças raras e da imunidade no Brasil. Material e métodos: Foram analisados retrospectivamente dados clínicos, radiológicos, patológicos e laboratoriais de pacientes com erros inatos da imunidade infectados por SARS-CoV-2 de março a dezembro de 2020. Resultados: Ao total, dados de 13 pacientes com diversos EII foram coletados para descrever tanto a evolução da doença quanto para buscar mais conhecimento sobre o tratamento desses pacientes. Em nenhum paciente a síndrome da angústia respiratória aguda foi observada, e também não foi observado nenhum óbito. A grande maioria dos pacientes teve evolução com síndrome gripal. Observou-se, em um paciente com CAPS-NLRP3, rash cutâneo vasculítico responsivo ao uso de anti-IL1. Conclusão: Neste pequeno grupo de pacientes com erros inatos da imunidade e com infecção por SARS-CoV-2, o risco de fatalidade foi menor do que observado na literatura. Especialmente, o fato de que a maioria apresenta maior predisposição a inflamação do que infecção deve ser levada em conta na análise dos dados finais. Reportamos pela primeira vez a presença de urticária vasculítica em paciente com CAPS, que habitualmente apresentam-se com urticária neutrofílica. Tal achado ressalta a capacidade de injúria vascular do vírus, mesmo em indivíduos predispostos geneticamente.

Introduction: The pandemic caused by the new coronavirus has become a global public health challenge. To date, many pathophysiological mechanisms of the virus have been explained, which is essential to understand clinical evolution and to develop appropriate therapeutic strategies for patients with COVID-19. However, less is known about COVID-19 in patients with inborn errors of immunity (IEI), especially in those with autoinflammatory disorders. Objective: To report the natural evolution of a group of patients with IEI infected with SARS-CoV-2 treated at a center of excellence in rare diseases and immunity in Brazil. Material and methods: Clinical, radiological, pathological, and laboratory data of patients treated from March to December 2020 were retrospectively retrieved and analyzed. Results: Data of 13 patients with IEI were collected to describe the natural course of the infection with SARS-CoV-2 and to enhance understanding of treatment for these patients. Neither acute respiratory distress syndrome nor death were observed. The vast majority of patients had flu-like symptoms. Urticarial vasculitis was observed in one patient with CAPS-NLRP3 responsive to the use of anti-IL1. Conclusion: In this small group of patients with IEI and SARSCoV- 2 infection, fatality risk was lower than that observed in the literature. Importantly, the fact that our group is composed mainly of patients with predisposition to inflammation instead of infection should be taken into account for final data analysis. Furthermore, we observed for the first time the presence of urticarial vasculitis in a patient with CAPS, which is usually characterized by neutrophilic urticaria. Such finding reinforces the virus ability to cause vascular injury, even in individuals with a genetic predisposition.

Urticária e COVID-19: foco nesta manifestação clínica / Urticaria and COVID-19: focus on this clinical manifestation

Introdução: A urticária é uma doença caracterizada pelo desenvolvimento de urticas, angioedema ou ambos. Convencionalmente a urticária pode ser dividida, quanto a sua duração, em duas formas: aguda (UA), quando os sintomas duram menos de seis semanas, e crônica (UC), com seis semanas ou mais de evolução. A COVID-19, enfermidade causada pelo coronavírus SARSCoV- 2, foi inicialmente descrita no final de 2019. A doença se apresenta por sintomas gripais, pneumonia, síndrome respiratória aguda grave e diarreia. Entretanto, o conhecimento atual sugere que a doença seja considerada sistêmica. Objetivo: Descrever as características dos pacientes que apresentaram UA ou exacerbação de UC durante infecção por COVID-19, atendidos em um Centro de Referência e Excelência em Urticária (GA2LEN UCARE). Métodos: De março a agosto de 2020 foram atendidos 12 pacientes com UA ou exacerbação da UC, diagnosticados com COVID-19. Resultados: Dentre os doze pacientes, 11 (92%) eram femininos. Quatro (33%) apresentaram UA entre o 1-6° dia da doença. Oito pacientes (67%) apresentaram exacerbação de UC, precedendo sintomas da COVID-19. Dentre estes, 5 (71%) apresentaram angioedema. Um aspecto importante foi o curso benigno destes pacientes, sem necessidade de hospitalização. Conclusão: Apesar da COVID-19 definir-se por doença respiratória, é essencial o olhar atento e criterioso para outras manifestações clínicas, como as cutâneas, que podem se apresentar como sintomas isolados ou associados. A identificação desta condição pode levar a uma melhoria no diagnóstico e terapia da COVID-19, bem como a uma aplicação mais rápida de práticas de quarentena.

Introduction: Urticaria is a disease characterized by the development of wheals, angioedema, or both. Conventionally, urticaria can be divided, on the basis of duration, into two forms: acute (AU), when symptoms last less than 6 weeks, and chronic (CU), when they last 6 weeks or more. COVID-19, a disease caused by the SARS-CoV-2 coronavirus, was first described in late 2019. The disease may manifest as flu-like symptoms, pneumonia, severe acute respiratory syndrome, and diarrhea. However, current knowledge suggests that the disease is systemic. Objective: To describe the characteristics of patients who presented with AU or exacerbation of CU during infection, treated at a Urticaria Center of Referral and Excellence (GA2LEN UCARE). Methods: From March to August 2020, 12 patients with AU or CU exacerbation who were diagnosed with COVID-19 were treated. Results: Among the 12 patients, 11 (92%) were female. Four patients (33%) had AU between days 1 and 6 of the disease. Eight patients (67%) had CU exacerbation preceding symptoms of COVID-19. Among these, 5 (71%) had angioedema. An important aspect was the benign course of these patients, with no need for hospitalization. Conclusion: Although COVID-19 is defined by respiratory disease, it is essential to look carefully for other clinical manifestations, such as cutaneous symptoms, which can be isolated or associated. The identification of this condition can lead to an improvement in the diagnosis and therapy of COVID-19, as well as a faster application of quarantine practices.

Evaluation of serum levels of C-reactive protein, D-Dimer and Autologous Serum Skin Test in patients with Chronic Spontaneous Urticaria in a Brazilian tertiary center: a cross-sectional study

Abstract Background: The pathophysiology of urticaria is still poorly understood. Recent studies demonstrate that the activation of coagulation is correlated with the clinical activity of Chronic Spontaneous Urticaria. Coagulation and inflammation are strongly linked. Objectives: To correlate the severity and activity of Chronic Spontaneous Urticaria with the levels of D-dimer, C-reactive protein, and autologous serum test in patients with Chronic Spontaneous Urticaria. Methods: The study included 55 patients diagnosed with chronic spontaneous urticaria. D-dimer levels were measured using enzyme-linked fluorescent assay and C-reactive protein levels were measured using the nephelometric method; autologous serum testing was performed on patients who discontinued antihistamine therapy. The severity of the disease was assessed using the urticaria activity score. Results: patients with severe, spontaneous, and difficult-to-control chronic urticaria had elevated serum levels of D-dimer, as well as a positive autologous serum test. Little correlation was demonstrated between the severity of chronic spontaneous urticaria and the levels of C-reactive protein. Conclusion: The authors concluded that patients with severe Chronic Spontaneous Urticaria showed signs of activated fibrinolysis. Most patients with high clinical scores had high D-dimer values. Patients with positive results for the autologous serum test also had more severe Chronic Spontaneous Urticaria and needed more drugs to control the disease. Finally, little correlation was found between C-reactive protein levels and disease severity. Study limitations: The main limitation was the small sample of patients. In the present patients, it was demonstrated that serum D-dimer levels and the autologous serum test can act as predictive markers of severity and activity of Chronic Spontaneous Urticaria.

Prolonged luteal phase support with progesterone may increase papules and plaques of pregnancy frequency in pregnancies through in vitro fertilization

Abstract Background: Pruritic urticarial papules and plaques of pregnancy development may have a strong relationship with hormone treatments during in vitro fertilization and hormonal changes during pregnancy. Objectives: The aim of this study was to evaluate and compare the frequency of papules and plaques of pregnancy and related factors in in vitro fertilization pregnancies and spontaneous pregnancies. Methods: In this study, 517 in vitro fertilization pregnancies and 1253 spontaneous pregnancies were retrospectively reviewed for papules and plaques of pregnancy frequency. The diagnosis of papules and plaques of pregnancy was performed by referral to the dermatology department and according to the typical clinical manifestations of the disease. Results: The papules and plaques of pregnancy was more common in all in vitro fertilization pregnancies (including single pregnancies) than in spontaneous pregnancies. Age, Rh positivity, mother weight gain, onset of disease during gestation, duration of disease, birth weight and the frequency of male fetus were similar between the two groups (p > 0.05). The rate of multiple pregnancies was higher in in vitro fertilization pregnancies with papules and plaques of pregnancy than in vitro fertilization pregnancies without papules and plaques of pregnancy (p < 0.001). Duration of progesterone treatment was also significantly longer in in vitro fertilization pregnancies with papules and plaques of pregnancy compared to in vitro fertilization pregnancies without papules and plaques of pregnancy (p < 0.001). Study limitations: The limitations of the study were the retrospective and single-centered design. Conclusion: The results of this study indicate that increased progesterone dosage or prolonged treatment may play a role in the pathogenesis papules and plaques of pregnancy.