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- Title
Response of Intractable Skin Ulcers in Recessive Dystrophic Epidermolysis Bullosa Patients to an Allogeneic Cultured Dermal Substitute.
- Authors
Natsuga, Ken; Sawamura, Daisuke; Goto, Maki; Homma, Erina; Goto-Ohguchi, Yuka; Aoyagi, Satoru; Akiyama, Masashi; Kuroyanagi, Yoshimitsu; Shimizu, Hiroshi
- 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.
- Subjects
SKIN disease treatment; ULCER treatment; EPIDERMOLYSIS bullosa; ELECTRON microscopy; EXTRACELLULAR matrix proteins; TRANSDERMAL medication; GRAFT versus host disease; THERAPEUTICS
- Publication
Acta Dermato-Venereologica, 2010, Vol 90, Issue 2, p165
- ISSN
0001-5555
- Publication type
Article
- DOI
10.2340/00015555-0776