{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T17:03:29Z","timestamp":1773939809559,"version":"3.50.1"},"reference-count":99,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T00:00:00Z","timestamp":1773878400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background\/Objectives: Cutaneous manifestations of inborn errors of immunity (IEI) are among the most common and often early signs of these disorders, estimated to affect about 40% of patients with IEI, and in some cases, they provide the first diagnostic clue. Skin findings in IEI are heterogeneous and include recurrent skin infections, severe atopic dermatitis, autoimmune manifestations, as well as atypical granulomatous dermatoses, neoplastic lesions, pigmentation disorders, and changes involving hair and nails. Early recognition of these manifestations and linking them to the appropriate immunologic defect is crucial for establishing the diagnosis and initiating targeted therapy. Methods: This paper reviews the dermatologic phenotypes associated with IEI, with particular emphasis on a tabular classification of skin lesions corresponding to specific immunologic defects. Relevant literature was analyzed to summarize characteristic cutaneous presentations and current diagnostic approaches, highlighting the importance of interdisciplinary evaluation. Results: Cutaneous findings in IEI encompass a wide spectrum of infectious, inflammatory, autoimmune, and neoplastic manifestations. Systematic classification of these lesions facilitates earlier recognition of underlying immune defects and supports differential diagnosis. Dermatologic signs frequently precede systemic manifestations, making them valuable early clinical indicators of IEI. Conclusions: Recognition of dermatologic manifestations is critical for early diagnosis of IEI. Interdisciplinary collaboration between dermatologists, immunologists, and other specialists improves diagnostic accuracy and patient management. Current therapeutic strategies range from symptomatic treatment to targeted therapies, and personalized approaches improve prognosis and quality of life in patients with IEI.<\/jats:p>","DOI":"10.3390\/medicina62030581","type":"journal-article","created":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T15:38:01Z","timestamp":1773934681000},"page":"581","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Cutaneous Manifestations of Inborn Errors of Immunity: Clinical Clues to Immune Disorders"],"prefix":"10.3390","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2202-3222","authenticated-orcid":false,"given":"Katarzyna","family":"Napiorkowska-Baran","sequence":"first","affiliation":[{"name":"Department of Allergology, Clinical Immunology and Internal Diseases, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University, 85-168 Torun, Poland"},{"name":"Department of Dermatology, Medical University of Silesia, 40-027 Katowice, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maciej","family":"Pastuszczak","sequence":"additional","affiliation":[{"name":"Department of Allergology, Clinical Immunology and Internal Diseases, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University, 85-168 Torun, Poland"},{"name":"Department of Dermatology, Medical University of Silesia, 40-027 Katowice, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7855-6215","authenticated-orcid":false,"given":"Maria","family":"P\u0142ocka-Karpi\u0144ska","sequence":"additional","affiliation":[{"name":"Academia Copernicana Interdisciplinary Doctoral School, Nicolaus Copernicus University, 87-100 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marta","family":"Tykwi\u0144ska","sequence":"additional","affiliation":[{"name":"Department of Allergology, Clinical Immunology and Internal Diseases, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University, 85-168 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8407-4642","authenticated-orcid":false,"given":"Pawe\u0142","family":"Treichel","sequence":"additional","affiliation":[{"name":"Doctoral School of Medical and Health Sciences, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University, 85-067 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gary Andrew","family":"Margossian","sequence":"additional","affiliation":[{"name":"School of Medicine Faculty, New York Medical College, Valhalla, NY 10595, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-2256-2752","authenticated-orcid":false,"given":"Carla Liana","family":"Margossian","sequence":"additional","affiliation":[{"name":"Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY 14203, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-2983-468X","authenticated-orcid":false,"given":"Agnieszka","family":"Rogalska","sequence":"additional","affiliation":[{"name":"Jan Biziel University Hospital No. 2, 85-168 Bydgoszcz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3418-1252","authenticated-orcid":false,"given":"Rafa\u0142","family":"Czajkowski","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology, Faculty of Medicine, Ludwik Rydygier Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University, 85-094 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,3,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"3030","DOI":"10.1016\/j.jaip.2023.06.037","article-title":"Cutaneous Findings in Inborn Errors of Immunity: An Immunologist\u2019s Perspective","volume":"11","author":"Cagdas","year":"2023","journal-title":"J. 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