Literature DB >> 18304158

Gerodermia osteodysplastica/wrinkly skin syndrome: report of three patients and brief review of the literature.

Arti Nanda1, Qasem A Alsaleh, Humoud Al-Sabah, Emad E Marzouk, Amr M A Salam, Mousumee Nanda, Jehoram T Anim.   

Abstract

Gerodermia osteodysplastica and wrinkly skin syndrome are rare autosomal recessive disorders. Due to the many phenotypic similarities in these two conditions, it has been proposed that they represent the same disorder. Both conditions are well delineated in the genetic literature, but despite skin involvement being a striking feature, they are rarely reported in dermatology journals. In this report, we describe three Arab children from two consanguineous families who exhibit overlapping features of gerodermia osteodysplastica and wrinkly skin syndrome. All the patients had dysmorphic facial features, wrinkled skin more marked on the hands and feet, hyperextensible joints, intrauterine growth retardation, developmental delay, congenital dislocation of hips, and osteoporosis. Our observations also support the contention that gerodermia osteodysplastica and wrinkly skin syndrome have the same clinical spectrum; however, this needs to be confirmed at the molecular level.

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Year:  2008        PMID: 18304158     DOI: 10.1111/j.1525-1470.2007.00586.x

Source DB:  PubMed          Journal:  Pediatr Dermatol        ISSN: 0736-8046            Impact factor:   1.588


  7 in total

1.  Lessons from cutis laxa syndromes: wrinkles due to improper reloading of the extracellular matrix?

Authors:  Uwe Kornak
Journal:  Eur J Hum Genet       Date:  2009-04-29       Impact factor: 4.246

2.  Mutation in pyrroline-5-carboxylate reductase 1 gene in families with cutis laxa type 2.

Authors:  Duane L Guernsey; Haiyan Jiang; Susan C Evans; Meghan Ferguson; Makoto Matsuoka; Mathew Nightingale; Andrea L Rideout; Sylvie Provost; Karen Bedard; Andrew Orr; Marie-Pierre Dubé; Mark Ludman; Mark E Samuels
Journal:  Am J Hum Genet       Date:  2009-07-02       Impact factor: 11.025

3.  Examining tissue composition, whole-bone morphology and mechanical behavior of GorabPrx1 mice tibiae: A mouse model of premature aging.

Authors:  Haisheng Yang; Laia Albiol; Wing-Lee Chan; Dag Wulsten; Anne Seliger; Michael Thelen; Tobias Thiele; Lyudmila Spevak; Adele Boskey; Uwe Kornak; Sara Checa; Bettina M Willie
Journal:  J Biomech       Date:  2017-10-25       Impact factor: 2.712

4.  Congenital Cutis Laxa Type 2 Associated With Recurrent Aspiration Pneumonia and Growth Delay: Case Report.

Authors:  Mohammadbagher Rahmati; Maryam Yazdanparast; Keramatallah Jahanshahi; Mohadese Zakeri
Journal:  Electron Physician       Date:  2015-10-19

5.  Cutis laxa autosomal recessive type II or wrinkly skin syndrome?

Authors:  Pooja Arora; Payal Chakravarty; Deepshikha Khanna; Ruchika Gupta
Journal:  Indian Dermatol Online J       Date:  2016 Sep-Oct

Review 6.  Discriminative Features in Three Autosomal Recessive Cutis Laxa Syndromes: Cutis Laxa IIA, Cutis Laxa IIB, and Geroderma Osteoplastica.

Authors:  Ariana Kariminejad; Fariba Afroozan; Bita Bozorgmehr; Alireza Ghanadan; Susan Akbaroghli; Hamid Reza Khorram Khorshid; Faezeh Mojahedi; Aria Setoodeh; Abigail Loh; Yu Xuan Tan; Nathalie Escande-Beillard; Fransiska Malfait; Bruno Reversade; Thatjana Gardeitchik; Eva Morava
Journal:  Int J Mol Sci       Date:  2017-03-15       Impact factor: 5.923

7.  Mutations in PYCR1 cause cutis laxa with progeroid features.

Authors:  Bruno Reversade; Nathalie Escande-Beillard; Aikaterini Dimopoulou; Björn Fischer; Serene C Chng; Yun Li; Mohammad Shboul; Puay-Yoke Tham; Hülya Kayserili; Lihadh Al-Gazali; Monzer Shahwan; Francesco Brancati; Hane Lee; Brian D O'Connor; Mareen Schmidt-von Kegler; Barry Merriman; Stanley F Nelson; Amira Masri; Fawaz Alkazaleh; Deanna Guerra; Paola Ferrari; Arti Nanda; Anna Rajab; David Markie; Mary Gray; John Nelson; Arthur Grix; Annemarie Sommer; Ravi Savarirayan; Andreas R Janecke; Elisabeth Steichen; David Sillence; Ingrid Hausser; Birgit Budde; Gudrun Nürnberg; Peter Nürnberg; Petra Seemann; Désirée Kunkel; Giovanna Zambruno; Bruno Dallapiccola; Markus Schuelke; Stephen Robertson; Hanan Hamamy; Bernd Wollnik; Lionel Van Maldergem; Stefan Mundlos; Uwe Kornak
Journal:  Nat Genet       Date:  2009-08-02       Impact factor: 38.330

  7 in total

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