Document Detail


Donor dominance cures CHILD nevus.
MedLine Citation:
PMID:  20389027     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Congenital hemidysplasia with ichthyosiform nevus and limb defects (MIM 308050, CHILD) syndrome is an X-linked dominant, male-lethal, multisystem birth defect. Patients suffer from an inflammatory nevus that covers large areas, predominantly of one side of the body, with a sharp midline demarcation. Treatment of CHILD nevus is notoriously difficult. OBJECTIVE: The aim of this study was to develop a novel surgical approach for this disorder. METHODS: In 2 patients, the CHILD nevus was dermabraded, and the area was covered with split skin grafts obtained from a contralateral unaffected donor region. In a third patient, papillomatous, strawberry-like lesions on fingers and toes were excised, and the defects were covered with full-thickness grafts obtained from the unaffected left, gluteal area. RESULTS: Highly satisfying functional and cosmetic results were documented during a follow-up period ranging from 3 to 8 years. CONCLUSION: The favorable outcome, superior to that obtained by simple dermabrasion or extensive plastic surgery, can best be explained by the donor dominance of the grafted skin samples that carried, in all or most cells, the mutant X chromosome in an inactivated form.
Authors:
Arne König; Jan Skrzypek; Harald Löffler; Frank Oeffner; Karl-Heinz Grzeschik; Rudolf Happle
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-04-09
Journal Detail:
Title:  Dermatology (Basel, Switzerland)     Volume:  220     ISSN:  1421-9832     ISO Abbreviation:  Dermatology (Basel)     Publication Date:  2010  
Date Detail:
Created Date:  2010-06-30     Completed Date:  2010-10-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9203244     Medline TA:  Dermatology     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  340-5     Citation Subset:  IM    
Copyright Information:
Copyright (c) 2010 S. Karger AG, Basel.
Affiliation:
Department of Dermatology, Philipp University, Deutschhausstrasse 9, Marburg, Germany.
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MeSH Terms
Descriptor/Qualifier:
3-Hydroxysteroid Dehydrogenases / genetics
Adult
Child
Codon, Nonsense
Female
Fingers
Genetic Diseases, X-Linked / surgery*
Humans
Ichthyosiform Erythroderma, Congenital / surgery*
Limb Deformities, Congenital / surgery*
Nevus / surgery*
Skin Neoplasms / surgery*
Skin Transplantation / methods
Syndrome
Treatment Outcome
Chemical
Reg. No./Substance:
0/Codon, Nonsense; EC 1.1.-/3-Hydroxysteroid Dehydrogenases; EC 1.1.1.170/Nsdhl protein, human

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