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Genetic inheritance has previously been reported as autosomal dominant or recessive with both forms harboring mutations in <jats:italic>COL7A1<\/jats:italic>. To date, there have been no reports of this rare clinical entity from various Southeast Asian ethnicities. Here, we describe the clinical and molecular features of five patients from the Southeast Asia region who presented with predominantly acral\u2010distributed blisters and erosions in the first few days of life. Blistering resolved over several months, without appearance of new blisters. By immunofluorescence, intraepidermal retention of Type VII collagen was observed in all patient skin biopsies when investigated with antibody staining. Genetic analysis of four patients revealed pathogenic variants in <jats:italic>COL7A1<\/jats:italic> which have not been previously reported. The clinical diagnosis in these rare patients is confirmed with molecular histology and genetic characterization.<\/jats:p>","DOI":"10.1002\/ajmg.a.61975","type":"journal-article","created":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T07:52:50Z","timestamp":1606809170000},"page":"625-630","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Self\u2010improving dystrophic epidermolysis bullosa: First report of clinical, molecular, and genetic characterization of five patients from Southeast Asia"],"prefix":"10.1002","volume":"185","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5685-4329","authenticated-orcid":false,"given":"Priya","family":"Bishnoi","sequence":"first","affiliation":[{"name":"Dermatology Service KK Women's &amp; Children's Hospital  Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi Zhen","family":"Ng","sequence":"additional","affiliation":[{"name":"Skin Research Institute of Singapore, Agency for Science, Technology and Research (A*STAR)  Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heming","family":"Wei","sequence":"additional","affiliation":[{"name":"Research Laboratory KK Women's &amp; Children's Hospital  Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5497-6058","authenticated-orcid":false,"given":"Ene\u2010Choo","family":"Tan","sequence":"additional","affiliation":[{"name":"Research Laboratory KK Women's &amp; Children's Hospital  Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Declan P.","family":"Lunny","sequence":"additional","affiliation":[{"name":"Skin Research Institute of Singapore, Agency for Science, Technology and Research (A*STAR)  Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"X. 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Colin C.","family":"Wong","sequence":"additional","affiliation":[{"name":"Skin Research Institute of Singapore, Agency for Science, Technology and Research (A*STAR)  Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leong","family":"Kin Fon","sequence":"additional","affiliation":[{"name":"Pediatric institute, Kuala Lumpur General Hospital  Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2327-2773","authenticated-orcid":false,"given":"Srie Prihianti","family":"Gondokaryono","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology, Faculty of Medicine Universitas Padjadjaran, Dr. Hasan Sadikin General Hospital  Bandung Indonesia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inne Arline","family":"Diana","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology, Faculty of Medicine Universitas Padjadjaran, Dr. Hasan Sadikin General Hospital  Bandung Indonesia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John E. 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