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Clinical findings include a tight, thin, translucent, taut skin, which tears spontaneously in flexion creases, arthrogryposis multiplex congenita (including the temporomandibular joint), enlarged fontanelles, typical face and dysplasia of clavicles and long bones. Histologic abnormalities include hyperplastic, abnormally keratinized epidermis, reduced tonofilaments, thin, compact dermis with hypoplasia of the elastic fibres, and abnormal subcutaneous fat. Fifteen previous cases are reviewed. \u00a9 1992 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.1320430308","type":"journal-article","created":{"date-parts":[[2005,6,12]],"date-time":"2005-06-12T04:42:21Z","timestamp":1118551341000},"page":"539-547","source":"Crossref","is-referenced-by-count":39,"title":["Restrictive dermopathy, a lethal form of arthrogryposis multiplex with skin and bone dysplasias: Three new cases and review of the literature"],"prefix":"10.1002","volume":"43","author":[{"given":"Alain","family":"Verloes","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicole","family":"Mulliez","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marie","family":"Gonzales","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fran\u00e7oise","family":"Laloux","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Trinh","family":"Hermanns\u2010L\u011b","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00e9rald E.","family":"Pi\u00e9rard","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lucien","family":"Koulischer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,6,7]]},"reference":[{"key":"e_1_2_1_2_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","volume":"93","author":"Bart BJ","year":"1966","journal-title":"Arch Dermatol"},{"key":"e_1_2_1_3_1","first-page":"116","article-title":"Aplasia cutis congenita\u2014the Carmi syndrome: Confirmation of a new neonatal generalised skin disorder","volume":"2","author":"Carey JC","year":"1983","journal-title":"Proc Greenwood Genet Center"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320110308"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(78)80436-3"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320270131"},{"key":"e_1_2_1_7_1","unstructured":"HamelBCJ KolleLAA NijhuisJG Anton\u2010LamprechtI(1990)Recesive dermopathy: A false\u2010negative prenatal diagnosis. 1st European Meeting of Dysmorphology Zaragossa September 10\u201311 1990."},{"key":"e_1_2_1_8_1","unstructured":"HappleR(1989)Restrictive dermopathy 5th International Congress of Paediatric Dermatology Milan 11\u201315 July."},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12466219"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0028-2243(80)90023-4"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320220311"},{"key":"e_1_2_1_12_1","volume-title":"Mendelian Inheritance in Man","author":"McKusick VA","year":"1990"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.27.5.315"},{"key":"e_1_2_1_14_1","unstructured":"OpitzJM(1988) Pathogenetic analysis of certain developmental defects. 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