{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,7]],"date-time":"2025-10-07T11:37:51Z","timestamp":1759837071717,"version":"3.37.3"},"reference-count":28,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2023,5,16]],"date-time":"2023-05-16T00:00:00Z","timestamp":1684195200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,7,21]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Epidermolysis bullosa (EB) is a devastating genetic condition caused by mutations in genes that give rise to aberrant proteins. There are 16 different such proteins implicated in EB that are important in maintaining the integrity of the dermoepidermal junction. It is classified into four major subtypes: (i) EB simplex; (ii) junctional EB (JEB); (iii) dystrophic EB (DEB); and (iv) Kindler EB. Renal disease is a recognized complication of EB and the aetiology is complex. We describe our experience of managing five patients with EB and IgA nephropathy. We recommend that patients with recessive DEB and JEB routinely have the following monitored: renal function, urinary albumin\/creatinine ratio, urine analysis, serum albumin levels and immunoglobulins; specifically serum IgA. Management of IgA nephropathy in the context of EB should be tailored to the individual and be carried out within a specialist multidisciplinary team. Our case series provides important insights into the treatment of IgA nephropathy in patients with EB and will help inform treatment in this rare genetic disease. Case series and reports like ours are key in gaining real-life data to quantify the actual risk of morbidity and mortality from each of the treatment modalities discussed.<\/jats:p>","DOI":"10.1093\/ced\/llad091","type":"journal-article","created":{"date-parts":[[2023,5,16]],"date-time":"2023-05-16T18:23:52Z","timestamp":1684261432000},"page":"920-925","source":"Crossref","is-referenced-by-count":1,"title":["IgA nephropathy in adults with epidermolysis bullosa"],"prefix":"10.1093","volume":"48","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4620-6082","authenticated-orcid":false,"given":"Manrup K","family":"Hunjan","sequence":"first","affiliation":[{"name":"National Adult Epidermolysis Bullosa Unit, Solihull Hospital, University Hospitals Birmingham NHS Foundation Trust , UK"},{"name":"College of Medical and Dental Sciences, University of Birmingham , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ajoy","family":"Bardhan","sequence":"additional","affiliation":[{"name":"National Adult Epidermolysis Bullosa Unit, Solihull Hospital, University Hospitals Birmingham NHS Foundation Trust , UK"},{"name":"Institute of Clinical Sciences, College of Medical and Dental Sciences, University of Birmingham , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natasha","family":"Harper","sequence":"additional","affiliation":[{"name":"National Adult Epidermolysis Bullosa Unit, Solihull Hospital, University Hospitals Birmingham NHS Foundation Trust , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dario Leonardo","family":"Balacco","sequence":"additional","affiliation":[{"name":"Institute of Clinical Sciences, College of Medical and Dental Sciences, University of Birmingham , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gerald","family":"Langman","sequence":"additional","affiliation":[{"name":"Departments of Cellular Pathology"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vijay","family":"Suresh","sequence":"additional","affiliation":[{"name":"Renal Medicine, University Hospitals Birmingham NHS Foundation Trust , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adrian","family":"Heagerty","sequence":"additional","affiliation":[{"name":"National Adult Epidermolysis Bullosa Unit, Solihull Hospital, University Hospitals Birmingham NHS Foundation Trust , UK"},{"name":"Institute of Ageing and Inflammation, University of Birmingham , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2023,5,16]]},"reference":[{"key":"2023101015422747100_llad091-B1","first-page":"78","article-title":"Epidermolysis bullosa","volume-title":"Nat Rev Dis 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