Médecine

Paramédical

Autres domaines


S'abonner

Graftskin therapy in epidermolysis bullosa - 29/08/11

Doi : 10.1067/mjd.2003.502 
David P. Fivenson, MDa, Lubomira Scherschun, MDa, Michelle Choucair, MDa, Debra KuKuruga, PhDb, Janet Young, PhDc, Tor Shwayder, MDa
Detroit, Michigan, and Canton, Massachusetts 
From the Departments of Dermatologya and Pathology,b Henry Ford Hospital, and Organogenesis Inc.c 

Abstract

Background: Epidermolysis bullosa (EB) is a family of 23 genetic skin disorders for which treatments are mainly supportive. Graftskin is a bilayered living human skin construct characterized by a normal expression profile of all the genes reported as mutant in EB. Objective: The objective of this study was to evaluate the efficiency and durability of graftskin in the treatment of EB. Methods: A total of 9 children with EB were treated with graftskin. These include EB simplex: Dowling-Meara type (n = 2); Weber-Cockayne type (n = 1); junctional EB-Herlitz type (n = 1); and recessive dystrophic EB (n = 5). Lesions were debrided of epidermis and crusts followed by application of fenestrated graftskin under sterile conditions. Syndactyly hand release for “mitten deformity” was performed after removal of all epidermis under general anesthesia. All treatment sites were dressed with a nonadherent contact layer followed by absorbent foam dressing, roll gauze, and a compression wrap covering and were left intact for 1 week. Graft take was assessed clinically at weeks 1, 2, 4, 12, and 20 to 28. Graft persistence was assessed by electron microscopy and polymerase chain reaction analysis at weeks 4 and 12, and between weeks 20 and 28 on selected cases. Results: A total of 96 sites were treated with 90% to 100% healing observed by 5 to 7 days, and many sites appearing as normal skin by 10 to 14 days. Finger and hand lesions showed 50% to 90% improvement in range of motion over baseline. Two children learned to walk after graftskin treatment of chronic plantar lesions. Two children had improvement in their chronic anemia after graftskin treatment. All patients and/or parents reported rapid pain resolution. Immunologic and genetic studies of graft persistence revealed evidence of donor DNA up to 28 weeks after graftskin application. None of the samples from female patients demonstrated Y chromosome-specific sequence when analyzed by the method of short tandem repeat. Conclusion: The encouraging results reported herein support the hypothesis that graftskin is more than a simple bandage or a source of growth factors to stimulate autologous closure of EB wounds. The improved quality of life and rapid achievement of growth/development milestones we have observed makes this an exciting step forward in the care of the patient with EB. (J Am Acad Dermatol 2003;48:886-92.)

Le texte complet de cet article est disponible en PDF.

Abbreviations : DM, EB, EBS, JEB, PCR, STR, WC


Plan


 Supported by clinical research grants from Novartis Pharmaceuticals Inc and Organogenesis Inc. Novartis Pharmaceuticals Inc also provided the funds for investigational studies and publication-related costs.
☆☆ Disclosure: Novartis Pharmaceuticals Inc is the marketing company for graftskin, and Organogenesis Inc is the maker of graftskin.
 Reprint requests: David P. Fivenson, MD, Henry Ford Health System, 2799 W Grand Blvd, Detroit, MI 48202. E-mail: dfivens1@hfhs.org.
★★ 0190-9622/2003/$30.00 + 0


© 2003  American Academy of Dermatology, Inc. Publié par Elsevier Masson SAS. Tous droits réservés.
Ajouter à ma bibliothèque Retirer de ma bibliothèque Imprimer
Export

    Export citations

  • Fichier

  • Contenu

Vol 48 - N° 6

P. 886-892 - juin 2003 Retour au numéro
Article précédent Article précédent
  • Lipid profile, insulin secretion, and insulin sensitivity in psoriasis
  • Claudia Reynoso-von Drateln, Esperanza Martínez-Abundis, Blanca Rebeca Balcázar-Muñoz, Rafael Bustos-Saldaña, Manuel González-Ortiz
| Article suivant Article suivant
  • Treatment of cutaneous leishmaniasis by photodynamic therapy
  • Kerstin Gardlo, Zuzana Horska, Claes David Enk, Lucie Rauch, Mossad Megahed, Thomas Ruzicka, Clemens Fritsch

Bienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.

Déjà abonné à cette revue ?

Mon compte


Plateformes Elsevier Masson

Déclaration CNIL

EM-CONSULTE.COM est déclaré à la CNIL, déclaration n° 1286925.

En application de la loi nº78-17 du 6 janvier 1978 relative à l'informatique, aux fichiers et aux libertés, vous disposez des droits d'opposition (art.26 de la loi), d'accès (art.34 à 38 de la loi), et de rectification (art.36 de la loi) des données vous concernant. Ainsi, vous pouvez exiger que soient rectifiées, complétées, clarifiées, mises à jour ou effacées les informations vous concernant qui sont inexactes, incomplètes, équivoques, périmées ou dont la collecte ou l'utilisation ou la conservation est interdite.
Les informations personnelles concernant les visiteurs de notre site, y compris leur identité, sont confidentielles.
Le responsable du site s'engage sur l'honneur à respecter les conditions légales de confidentialité applicables en France et à ne pas divulguer ces informations à des tiers.