{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T20:17:00Z","timestamp":1792268220094,"version":"4.4.0"},"reference-count":37,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2019,8,21]],"date-time":"2019-08-21T00:00:00Z","timestamp":1566345600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Photoderm Photoimm Photomed"],"published-print":{"date-parts":[[2020,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Erythropoietic protoporphyria (EPP) is a semi\u2010dominantly inherited porphyria presenting with photosensitivity during early childhood. Acquired EPP has been reported; however, data regarding this rare disorder are scarce.<\/jats:p><\/jats:sec><jats:sec><jats:title>Purpose<\/jats:title><jats:p>To evaluate the characteristics of acquired EPP.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A comprehensive search of PubMed, Google Scholar, ScienceDirect, and clinicaltrials.gov databases was performed by three reviewers. Studies describing patients with acquired EPP were included. Additionally, we present an index case of a 26\u2010year\u2010old patient who acquired clinically and biochemically typical EPP in association with myelodysplastic syndrome (MDS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We included 20 case reports describing 20 patients. Most (80%) patients were male of mean age 58\u00a0\u00b1\u00a013\u00a0years. In all patients, acquired EPP was associated with hematological disease, most commonly MDS (85%) followed by myeloproliferative disease (10%). In 86% of cases, hematological disease led to abnormality or somatic mutation in chromosome 18q (the locus of the ferrochelatase gene). The mean erythrocyte protoporphyrin IX concentration was very high (4286\u00a0\u03bcg\/dL). Most (90%) patients presented with photosensitivity, 20% experienced blistering, and 25% presented with hepatic insufficiency, both uncommon in EPP. In 55% of patients, hematological disease was diagnosed after occurrence of cutaneous symptoms. Beta\u2010carotene led to partial control of symptoms in 5 patients and resolution in another patient. Azacitidine treatment of MDS led to resolution of cutaneous symptoms in three patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We present the distinct features of acquired EPP and highlight that any patient presenting with new\u2010onset photosensitivity, irrespective of age should be evaluated for porphyria.<\/jats:p><\/jats:sec>","DOI":"10.1111\/phpp.12501","type":"journal-article","created":{"date-parts":[[2019,8,2]],"date-time":"2019-08-02T17:57:48Z","timestamp":1564768668000},"page":"29-33","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Acquired erythropoietic protoporphyria: A systematic review of the literature"],"prefix":"10.1111","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7126-2193","authenticated-orcid":false,"given":"Igor","family":"Snast","sequence":"first","affiliation":[{"name":"Photodermatosis Clinic, Department of Dermatology Rabin Medical Center Petach Tikva Israel"},{"name":"Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ran","family":"Kaftory","sequence":"additional","affiliation":[{"name":"Photodermatosis Clinic, Department of Dermatology Rabin Medical Center Petach Tikva Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shani","family":"Sherman","sequence":"additional","affiliation":[{"name":"Photodermatosis Clinic, Department of Dermatology Rabin Medical Center Petach Tikva Israel"},{"name":"Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yonatan","family":"Edel","sequence":"additional","affiliation":[{"name":"Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel"},{"name":"Porphyria Center Rabin Medical Center Petach Tikva Israel"},{"name":"Rheumatology Unit Rabin Medical Center Petach Tikva Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmilia","family":"Hodak","sequence":"additional","affiliation":[{"name":"Photodermatosis Clinic, Department of Dermatology Rabin Medical Center Petach Tikva Israel"},{"name":"Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Assi","family":"Levi","sequence":"additional","affiliation":[{"name":"Photodermatosis Clinic, Department of Dermatology Rabin Medical Center Petach Tikva Israel"},{"name":"Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moshe","family":"Lapidoth","sequence":"additional","affiliation":[{"name":"Photodermatosis Clinic, Department of Dermatology Rabin Medical Center Petach Tikva Israel"},{"name":"Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,8,21]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.15769"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.5041\/RMMJ.10333"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-4-19"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-2829"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1080\/08998280.2016.11929448"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.13709"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcms.2010.11.001"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/PRS.0b013e3181f44abc"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-013-1720-6"},{"key":"e_1_2_6_11_1","first-page":"1971","article-title":"A case of sideroblastic anemia with dermal photosensitivity and increased erythrocyte protoporphyrin","volume":"22","author":"Sato Y","year":"1981","journal-title":"Rinsho Ketsuek"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-009-0859-7"},{"key":"e_1_2_6_13_1","unstructured":"BottomleySS MooreMZ.Acquired erythropoietic protoporphyria and sideroblastic anemia. 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