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Comprehensive Assessment of Skin Disorders in Patients With Common Variable Immunodeficiency (Cvid) Publisher Pubmed



Zarezadeh Mehrabadi A1 ; Aghamohamadi N1 ; Abolhassani H2, 3 ; Aghamohammadi A2 ; Rezaei N2, 4 ; Yazdani R2, 4, 5
Authors
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Authors Affiliations
  1. 1. Immunology Department, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
  2. 2. Research Center for Immunodeficiencies, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Division of Clinical Immunology, Department of Bioscience and Nutrition, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden
  4. 4. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  5. 5. Department of Neurology, Thomas Jefferson University, Philadelphia, 19107, PA, United States

Source: Journal of Clinical Immunology Published:2022


Abstract

Background: Common variable immunodeficiency (CVID) is an inborn error of immunity (IEI) characterized by various clinical manifestations such as hypogammaglobulinemia, recurrent infections, and autoimmune diseases. Among different clinical manifestations, skin manifestations have been less reported in these patients. Methods: In this study, we investigated the prevalence of dermatologic features in 387 CVID patients. Demographic information, clinical manifestations, laboratory data, and genetic findings were collected from medical records. All data were analyzed based on the presence or absence of skin disorders in CVID patients. Results: We observed at least one skin manifestation in about 40% of these patients. Among these complications, skin infection (n = 64, 42.1%) was the most frequent presentation, followed by non-infectious skin lesions (n = 54, 35.6%). Among skin infections, abscesses (n = 34, 22.4%) were the most common complication. Skin infections such as cellulitis, impetigo, measles, and warts were also documented. Eczema (n = 34, 22.4%) was the most common complication in atopic lesions, and vitiligo (n = 13, 8.5%) was prevalent in autoimmune/pigmentation disorders. Among all the patients with genetic mutations, one-quarter had a deleterious mutation in the LRBA gene, relating to the autoimmune and atopic skin lesions. Conclusion: This rate of skin disorders in our cohort demonstrating these manifestations could be significant in CVID patients, and they are not rare. Low data of skin complications in CVID patients could be attributed to insufficient attention of physicians and also might alert dermatologists to perform immunological investigations in children with certain skin manifestations. © 2022, The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
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