Document Detail


Uncombable hair and atopic dermatitis in a case of tricho-dento-osseous syndrome.
MedLine Citation:
PMID:  19624803     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Summary The tricho-dento-osseous syndrome is a rare genetic disorder due to a mutation in the DLX3 gene on chromosome 17q21. It can affect hair, teeth, bone and nails, causing phenotypic variability. We report on an 8-year-old girl with tricho-dento-osseous syndrome, who presented in our allergy center with severe atopic dermatitis. Additional clinical findings included light, kinky hair reminiscent of pili trianguli et canaliculi (uncombable hair), enamel hypoplasia and enlarged pulp chambers of the molar teeth (tau-rodontism). A genetic investigation revealed a de-novo mutation in the DLX3 gene on chromosome 17q21. Electron microscopic examination of the curly hair showed a flattened hair shaft with longitudinal grooves.
Authors:
Dagmara E Y Mayer; Christian Baal; Margaritha Litschauer-Poursadrollah; Wolfgang Hemmer; Reinhart Jarisch
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2009-7-14
Journal Detail:
Title:  Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG     Volume:  -     ISSN:  1610-0387     ISO Abbreviation:  J Dtsch Dermatol Ges     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-7-23     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101164708     Medline TA:  J Dtsch Dermatol Ges     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
FAZ - Floridsdorf Allergy Center, Vienna, Austria.
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