Literature DB >> 8302735

Severe skin erosions and scalp infections in AEC syndrome.

S L Vanderhooft1, M J Stephan, V P Sybert.   

Abstract

Hay-Wells syndrome is an autosomal dominant condition characterized by ankyloblepharon filiforme adnatum, ectodermal dysplasia, and cleft palate with or without associated cleft lip (AEC syndrome). Although several reported patients had eroded skin at birth and recurrent scalp infections, these are not generally regarded as major features of the disorder. In our experience, denuded skin at birth and chronic scalp erosions complicated by infection are common features of this syndrome. Aggressive wound care in conjunction with early administration of topical or systemic antibiotics is suggested.

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Year:  1993        PMID: 8302735     DOI: 10.1111/j.1525-1470.1993.tb00394.x

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


  8 in total

1.  Management of ankyloblepharon filiforme adnatum.

Authors:  A Ioannides; N D Georgakarakos
Journal:  Eye (Lond)       Date:  2011-02-25       Impact factor: 3.775

2.  Case report: unusual dental morphology in a child with ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome.

Authors:  K Fitzgerald; S A Lynch; E McKiernan
Journal:  Eur Arch Paediatr Dent       Date:  2011-08

Review 3.  Integrating animal models and in vitro tissue models to elucidate the role of desmosomal proteins in diseases.

Authors:  Maranke I Koster; Jason Dinella; Jiangli Chen; Charlene O'Shea; Peter J Koch
Journal:  Cell Commun Adhes       Date:  2014-02

Review 4.  Modeling AEC-New approaches to study rare genetic disorders.

Authors:  Peter J Koch; Jason Dinella; Mary Fete; Elaine C Siegfried; Maranke I Koster
Journal:  Am J Med Genet A       Date:  2014-03-24       Impact factor: 2.802

5.  Rare Variant of Ankyloblepharon-ectodermal Defect-cleft Lip/Cleft Palate Syndrome: Curly Hair-ankyloblepharon-nail Disease Syndrome.

Authors:  Ajay Chopra; Debdeep Mitra; Renu Kandpal; Reetu Agarwal
Journal:  Int J Trichology       Date:  2018 Jan-Feb

6.  Gene p63: In ectrodactyly-ectodermal dysplasia clefting, ankyloblepharon-ectodermal dysplasia, Rapp-Hodgkin syndrome.

Authors:  Cornelia van Straten; Kurt-W Butow
Journal:  Ann Maxillofac Surg       Date:  2013-01

7.  Claudin-1 is a p63 target gene with a crucial role in epithelial development.

Authors:  Teresa Lopardo; Nadia Lo Iacono; Barbara Marinari; Maria L Giustizieri; Daniel G Cyr; Giorgio Merlo; Francesca Crosti; Antonio Costanzo; Luisa Guerrini
Journal:  PLoS One       Date:  2008-07-23       Impact factor: 3.240

8.  Ankyloblepharon filiforme adnatum: a case report.

Authors:  Bouchra Alami; Asmae Maadane; Rachid Sekhsoukh
Journal:  Pan Afr Med J       Date:  2013-05-08
  8 in total

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