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. Copyright (c) 2010 S. Karger AG, Basel.
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. Copyright (c) 2010 S. Karger AG, Basel.
Authors: Marie Morimoto; Christèle du Souich; Joanne Trinh; Keith W McLarren; Cornelius F Boerkoel; Glenda Hendson Journal: J Mol Histol Date: 2011-11-24 Impact factor: 2.611
Authors: Amy S Paller; Maurice A M van Steensel; Marina Rodriguez-Martín; Jennifer Sorrell; Candrice Heath; Debra Crumrine; Michel van Geel; Antonio Noda Cabrera; Peter M Elias Journal: J Invest Dermatol Date: 2011-07-14 Impact factor: 8.551