title: Response of intractable skin ulcers in recessive dystrophic epidermolysis bullosa patients to an allogeneic cultured dermal substitute.
abstract: Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited skin disorder caused by mutations in the COL7A1 gene, which encodes collagen VII (COL7). Skin ulcers in RDEB patients are sometimes slow to heal. We describe here the therapeutic response of intractable skin ulcers in two patients with generalized RDEB to treatment with an allogeneic cultured dermal substitute (CDS). Skin ulcers in both patients epithelialized by 3-4 weeks after this treatment. Immunohistochemical studies demonstrated that the COL7 expression level remained reduced with respect to the control skin and that it did not differ significantly between graft-treated and untreated areas. Electron microscopy showed aberrant anchoring fibrils beneath the lamina densa of both specimens. In conclusion, CDS is a promising modality for treatment of intractable skin ulcers in patients with RDEB, even though it does not appear to increase COL7 expression.
authors.affiliation: Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan. natsuga@med.hokudai.ac.jp
authors.forename: Ken
authors.initials: K
authors.lastname: Natsuga
authors.forename: Daisuke
authors.initials: D
authors.lastname: Sawamura
authors.forename: Maki
authors.initials: M
authors.lastname: Goto
authors.forename: Erina
authors.initials: E
authors.lastname: Homma
authors.forename: Yuka
authors.initials: Y
authors.lastname: Goto-Ohguchi
authors.forename: Satoru
authors.initials: S
authors.lastname: Aoyagi
authors.forename: Masashi
authors.initials: M
authors.lastname: Akiyama
authors.forename: Yoshimitsu
authors.initials: Y
authors.lastname: Kuroyanagi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shimizu
mesh_terms: D000328:Adult; D002478:Cells, Cultured; D024143:Collagen Type VII / Q000235:genetics / Q000378:metabolism; D062109:Dermatologic Surgical Procedures; D016108:Epidermolysis Bullosa Dystrophica / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D005260:Female; D005347:Fibroblasts / Q000637:transplantation*; D006801:Humans; D008875:Middle Aged; D009154:Mutation; D012867:Skin / Q000000981:diagnostic imaging / Q000378:metabolism; D016038:Skin Transplantation; D012883:Skin Ulcer / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D017768:Skin, Artificial; D013997:Time Factors; D023822:Tissue Engineering; D054457:Tissue Scaffolds; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D014463:Ultrasonography; D014945:Wound Healing
chemical_list: D024143:Collagen Type VII