{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,20]],"date-time":"2025-05-20T18:43:13Z","timestamp":1747766593962,"version":"3.37.3"},"reference-count":12,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,7,19]],"date-time":"2020-07-19T00:00:00Z","timestamp":1595116800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,7,19]],"date-time":"2020-07-19T00:00:00Z","timestamp":1595116800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Med Case Reports"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n<jats:title>Background<\/jats:title>\n<jats:p>Pemphigus vulgaris is an autoimmune disorder that involves intraepithelial blistering and sores of the skin and mucous membranes. The average age of onset is between 50 and 70\u2009years. Pemphigus rarely occurs in children. It correlates with the level of circulating autoantibodies; therapeutic plasma exchange is hypothesized to remove pathogenic autoantibodies, and this is necessary in refractory severe cases.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Case presentation<\/jats:title>\n<jats:p>A 14-year-old Asian girl came to our hospital with blisters and erosions all over her body and in the oral mucosa. She was diagnosed with pemphigus vulgaris by skin biopsy about 3\u2009months before hospitalization. She was admitted to the intensive care unit due to aggressively worsening symptoms, extensive lesions, dehydration, and electrolyte imbalance secondary to excess fluid loss from the skin wounds and sepsis secondary to infection of the exposed wounds. She did not respond to prednisone and azathioprine therapy but was successfully treated with plasmapheresis.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusion<\/jats:title>\n<jats:p>The purpose of this case report is to describe an aggressive presentation of pemphigus vulgaris, especially because the onset of the disease in our patient was at an early age. The disease rarely begins in childhood, and this case report highlights the importance of plasmapheresis as a useful intervention in patients with pemphigus vulgaris who are not responding to conventional therapy, taking into account that there is a paucity of studies showing the effectiveness of plasmapheresis in inducing partial or complete remission in young patients.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s13256-020-02421-w","type":"journal-article","created":{"date-parts":[[2020,7,18]],"date-time":"2020-07-18T23:02:41Z","timestamp":1595113361000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Aggressive refractory pemphigus vulgaris that responded to plasmapheresis: a\u00a0case report"],"prefix":"10.1186","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8412-1552","authenticated-orcid":false,"given":"Hiba Hasan","family":"Khaddour","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Diana","family":"Zaher","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Triak","family":"Kassem","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmad","family":"Hasan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,7,19]]},"reference":[{"key":"2421_CR1","volume-title":"Fitzpatrick\u2019s dermatology in general medicine","author":"LA Goldsmith","year":"2012","unstructured":"Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K. 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Plasmapheresis in refractory pemphigus vulgaris: revisiting an old treatment modality used in synchrony with pulse cyclophosphamide. Cutis. 2015;95(3):E17\u20139.","journal-title":"Cutis"},{"issue":"1","key":"2421_CR10","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1016\/S1473-0502(02)00095-2","volume":"28","author":"G Mazzi","year":"2003","unstructured":"Mazzi G, Raineri A, Zanolli FA, et al. Plasmapheresis therapy in pemphigus vulgaris and bullous pemphigoid. Transfus Apher Sci. 2003;28(1):13\u20138.","journal-title":"Transfus Apher Sci"},{"issue":"1","key":"2421_CR11","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1111\/j.1468-3083.2010.03703.x","volume":"25","author":"L Sagi","year":"2011","unstructured":"Sagi L, Baum S, Gendelman V, Trau H, Barzilai A. The role of therapeutic plasma exchange in pemphigus vulgaris. 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Written informed consent was obtained before the patient\u2019s participation.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient\u2019s mother for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"109"}}