{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,12]],"date-time":"2025-09-12T18:59:25Z","timestamp":1757703565241},"reference-count":12,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2015,4,9]],"date-time":"2015-04-09T00:00:00Z","timestamp":1428537600000},"content-version":"vor","delay-in-days":6095,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Journal of Dermatology"],"published-print":{"date-parts":[[1998,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Background: Bart's syndrome is one type of dominant dystrophic epidermolysis bullosa (EB). It is known that, in some familial cases of dominant dystrophic EB, the symptoms differ depending on the individual. We observed the way Bart's syndrome affected four generations in the same family. The proband was a newborn boy who showed congenital localized absence of skin (CLAS) and bullae on the anterior aspects of both legs. Histologically, the bullae were located subepidermally. The CLAS and bullae disappeared within 4 months after birth, leaving scars. His father retained scarring and scaling from the knees down along the anterior aspect of the legs, and the nails of the toes were either lacking or deformed. His paternal grandmother and great\u2010grandmother also presented deformed nails of the toes, although they had not had CLAS or bullae on the legs at birth. The individuals in this family thus showed some heterogeneity depending on the sex: blistering and CLAS were seen on the legs soon after birth in the male family members, but the female members did not share this pattern of symptoms, suggesting that the expression of symptoms may differ depending on the sex of the affected individual.<\/jats:p>","DOI":"10.1111\/j.1346-8138.1998.tb02447.x","type":"journal-article","created":{"date-parts":[[2015,4,10]],"date-time":"2015-04-10T08:34:51Z","timestamp":1428654891000},"page":"517-522","source":"Crossref","is-referenced-by-count":8,"title":["Clinical Phenotype of Bart's Syndrome Seen in a Family with Dominant Dystrophic Epidermolysis Bullosa"],"prefix":"10.1111","volume":"25","author":[{"given":"Shoji","family":"Wakasugi","sequence":"first","affiliation":[{"name":"Department of Dermatology Kumamoto University School of Medicine  Kumamoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kumiko","family":"Mizutari","sequence":"additional","affiliation":[{"name":"Department of Dermatology Kumamoto University School of Medicine  Kumamoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomomichi","family":"Ono","sequence":"additional","affiliation":[{"name":"Department of Dermatology Kumamoto University School of Medicine  Kumamoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,4,9]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(85)70168-5"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1987.tb02836.x"},{"key":"e_1_2_1_4_1","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1001\/archderm.1966.01600210032005","article-title":"Congenital localized absence of skin and associated abnormalities resembling epidermolysis bullosa: A new syndrome","volume":"93","author":"Bart BJ","year":"1966","journal-title":"Arch Dermatol"},{"key":"e_1_2_1_5_1","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1001\/archderm.1978.01640130045013","article-title":"A mechanobullous disease of the newborn: Bart's syndrome","volume":"114","author":"Smith SZ","year":"1978","journal-title":"Arch Dermatol"},{"key":"e_1_2_1_6_1","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1001\/archderm.1995.01690180037006","article-title":"Bart's syndrome: Ultrastructure and genetic linkage","volume":"131","author":"Zelickson B","year":"1995","journal-title":"Arch Dermatol"},{"key":"e_1_2_1_7_1","doi-asserted-by":"crossref","first-page":"1087","DOI":"10.1001\/archderm.1992.01680180081010","article-title":"Congenital localized absence of the skin as a manifestation of epidermolysis bullosa","volume":"128","author":"Kanzler MH","year":"1992","journal-title":"Arch Dermatol"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12658066"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.1994.8"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.91.9.3549"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12349293"},{"key":"e_1_2_1_12_1","doi-asserted-by":"crossref","first-page":"277","DOI":"10.2340\/0001555577277280","article-title":"Dominant dystrophic epidermolysis bullosa albopapuloidea Pasini\u2014Ultrastructural observations of albopapuloid lesions and a type VII collagen DNA polymorphism study of a family","volume":"77","author":"Nomura K","year":"1997","journal-title":"Acta Derm Venereal (Stockh)"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0168-9525(94)90212-7"}],"container-title":["The Journal of Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1346-8138.1998.tb02447.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1346-8138.1998.tb02447.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,28]],"date-time":"2023-10-28T08:56:24Z","timestamp":1698483384000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1346-8138.1998.tb02447.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,8]]},"references-count":12,"journal-issue":{"issue":"8","published-print":{"date-parts":[[1998,8]]}},"alternative-id":["10.1111\/j.1346-8138.1998.tb02447.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1346-8138.1998.tb02447.x","archive":["Portico"],"relation":{},"ISSN":["0385-2407","1346-8138"],"issn-type":[{"value":"0385-2407","type":"print"},{"value":"1346-8138","type":"electronic"}],"subject":[],"published":{"date-parts":[[1998,8]]}}}