Chronic urticaria and common variable immunodeficiency (CVID): an association to remember

Santos, Claudia Villatoro; Yacoub, Michael

doi:10.1136/bcr-2020-239733

Cited by 3 publications

(2 citation statements)

References 12 publications

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“…CSU and AD are not usually found among the most common clinical manifestations in CVID [19]; however, there are reports suggesting that the association might be underdiagnosed [20][21][22][23]. The prevalence of CSU has also been found to be higher in patients with SIgAD (4.9%) than in controls (0.9%) [18], and in up to 12% of patients with SIgMD [16].…”

Section: Discussionmentioning

confidence: 99%

Skin Manifestations in Patients with Selective Immunoglobulin E Deficiency

Picado

García-Herrera

Hernández‐Rodríguez

et al. 2022

JCM

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Selective immunoglobulin E deficiency (SIgED) is still an unrecognised primary immunodeficiency despite several observations supporting its existence. This study aimed to describe the skin manifestations associated with SIgED. We retrospectively assessed medical records of patients with SIgED, the diagnosis being based on serum IgE levels ≤2 Uk/L associated with normal serum levels of immunoglobulins G, M, and A. A total of 25 patients (24 female) with SIgED were included in the study. Eleven patients (44%) presented chronic spontaneous urticaria (CSU), five (20%) angioedema always associated with CSU, five erythema (20%), and six eczema (24%). Other, less frequent manifestations were lichen planus, anaphylactoid purpura, thrombocytopenic purpura, bullous pemphigoid, bullous pyoderma gangrenosum, and atypical skin lymphoproliferative infiltrate associated with reactive lymphadenopathy, chronic cholestasis, arthritis, and fibrosing mediastinitis. Fifteen patients (60%) had different types of associated autoimmune diseases, Hashimoto’s thyroiditis being the most frequent (n = 5, 20%), followed by arthritis (n = 4, 16%), autoimmune hepatitis, neutropenia, vitiligo, and Sjögren’s syndrome (n = 2, 8% each). Five malignancies were diagnosed in four patients (16%). An ultralow IgE serum level may be the only biomarker that reveals the presence of a dysregulated immune system in patients with a broad spectrum of skin manifestations.

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Section: Discussionmentioning

confidence: 99%

Skin Manifestations in Patients with Selective Immunoglobulin E Deficiency

Picado

García-Herrera

Hernández‐Rodríguez

et al. 2022

JCM

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“…In contrast, CSU and angioedema are not usually listed among the more common clinical manifestations in CVID [ 29 , 65 ]. However, some reports point out that we should not overlook the association of CSU with CVID [ 70 , 71 , 72 , 73 ]. CSU has been found in 4.9% of patients with SIgAD compared with 0.9% in controls [ 74 ], and in up to 12% of patients with SIgMD [ 75 ].…”

Section: Discussionmentioning

confidence: 99%

Spectrum of Disease Manifestations in Patients with Selective Immunoglobulin E Deficiency

Picado

Landazuri

Vlagea

et al. 2021

JCM

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Background: Selective IgE deficiency (SIgED) has been previously evaluated in selected patients from allergy units. This study investigates the effects of SIgED on the entire population in a hospital setting and sought to delineate in detail the clinical aspects of SIgED. Methods: A retrospective study of the data obtained from electronic medical records of 52 adult patients (56% female) with a mean age of 43 years and IgE levels of <2.0 kU/L with normal immunoglobulin (Ig) IgG, IgA, and IgM levels, seen at our hospital, without selection bias, from 2010 to 2019. Results: Recurrent upper respiratory infections were recorded in 18 (34.6%) patients, pneumonia was recorded in 16 (30.7%) patients, bronchiectasis was recorded in 16 (30.7%) patients, and asthma was recorded in 10 (19.2%) patients. Eighteen patients (34.6%) suffered autoimmune clinical manifestations either isolated (19%) or combining two or more diseases (15%), Hashimoto’s thyroiditis being the most frequent (19%), which was followed by arthritis (10%) and thrombocytopenia and/or neutropenia (5.7%). Other less frequent associations were Graves’ disease, primary sclerosing cholangitis, Sjögren’s syndrome, and autoimmune hepatitis. Eczematous dermatitis (15.3%), chronic spontaneous urticaria (17.3%), and symptoms of enteropathy (21%) were also highly prevalent. Thirty percent of patients developed malignancies, with non-Hodgkin lymphomas (13.4%) being the most prevalent. Conclusions: The clinical manifestations of SIgED encompass a variety of infectious, non-infectious complications, and malignancy. Since it cannot be ruled out that some type of selection bias occurred in the routine assessment of IgE serum Ievels, prospective studies are required to better characterize SIgED and to determine whether it should be added to the list of antibody deficiencies.

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Clinical characteristics of chronic spontaneous urticaria in patients with Common variable immune deficiency and hypogammaglobulinemia

Фролов

Латышева

Latysheva

et al. 2021

Russian Journal of Allergy

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Chronic spontaneous urticaria is a common disease that can be associated with various autoimmune, infectious (viral, bacterial, parasitic) and non-infectious inflammatory diseases, as well as occur in the symptom complex of other diseases, such as primary immunodeficiencies (PID). Currently, data about the features of the course of chronic spontaneous urticaria in patients with PID are accumulating. In this article we present two clinical cases of patients with common variable immune deficiency and one clinical case of a patient with hypogammaglobulinemia, suffering from chronic spontaneous urticaria. The article describes peculiar properties of IVIG replacement therapy at the course of urticaria in these patients. The discussion section presents data from the world literature and offers key provisions for further investigations.

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Chronic urticaria and common variable immunodeficiency (CVID): an association to remember

Cited by 3 publications

References 12 publications

Skin Manifestations in Patients with Selective Immunoglobulin E Deficiency

Skin Manifestations in Patients with Selective Immunoglobulin E Deficiency

Spectrum of Disease Manifestations in Patients with Selective Immunoglobulin E Deficiency

Clinical characteristics of chronic spontaneous urticaria in patients with Common variable immune deficiency and hypogammaglobulinemia

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