title: Donor dominance cures CHILD nevus.
abstract: 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.
  
  
  CONCLUSIONS
  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.affiliation: Department of Dermatology, Philipp University, Deutschhausstrasse 9, Marburg, Germany.
authors.forename: Arne
authors.initials: A
authors.lastname: König
authors.forename: Jan
authors.initials: J
authors.lastname: Skrzypek
authors.forename: Harald
authors.initials: H
authors.lastname: Löffler
authors.forename: Frank
authors.initials: F
authors.lastname: Oeffner
authors.forename: Karl-Heinz
authors.initials: KH
authors.lastname: Grzeschik
authors.forename: Rudolf
authors.initials: R
authors.lastname: Happle
mesh_terms: D015096:3-Hydroxysteroid Dehydrogenases / Q000235:genetics; D000328:Adult; D002648:Child; D018389:Codon, Nonsense; D005260:Female; D005385:Fingers; D040181:Genetic Diseases, X-Linked / Q000601:surgery*; D006801:Humans; D016113:Ichthyosiform Erythroderma, Congenital / Q000601:surgery*; D017880:Limb Deformities, Congenital / Q000601:surgery*; D009506:Nevus / Q000601:surgery*; D012878:Skin Neoplasms / Q000601:surgery*; D016038:Skin Transplantation / Q000379:methods; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D018389:Codon, Nonsense; D015096:3-Hydroxysteroid Dehydrogenases; C478776:Nsdhl protein, human