Biomaterial Database

Epidermolysis bullosa: prospects for cell-based therapies. 2008

Jouni Uitto

Department of Dermatology and Cutaneous Biology, Jefferson Medical College, and Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA. Jouni.Uitto@jefferson.edu

Heritable forms of epidermolysis bullosa (EB) are characterized by chronic, lifelong blistering and erosions due to mutations in 10 distinct genes expressed at the cutaneous basement membrane zone. No specific treatment for this group of intractable diseases is currently available. Recent progress in molecular therapies has indicated that cell-based approaches may potentially offer amelioration--and perhaps even cure--for afflicted individuals. In this issue, Wong et al. (2008) demonstrate the feasibility of direct intradermal injection of allogeneic fibroblasts to the lesional skin of patients with recessive dystrophic EB, with improvement in skin fragility.

UI	MeSH Term	Description	Entries
D004820	Epidermolysis Bullosa	Group of genetically determined disorders characterized by the blistering of skin and mucosae. There are four major forms: acquired, simple, junctional, and dystrophic. Each of the latter three has several varieties.	Acantholysis Bullosa
D005347	Fibroblasts	Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules.	Fibroblast
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001706	Biopsy	Removal and pathologic examination of specimens from the living body.	Biopsies
D012867	Skin	The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D014184	Transplantation, Homologous	Transplantation between individuals of the same species. Usually refers to genetically disparate individuals in contradistinction to isogeneic transplantation for genetically identical individuals.	Transplantation, Allogeneic,Allogeneic Grafting,Allogeneic Transplantation,Allografting,Homografting,Homologous Transplantation,Grafting, Allogeneic
D024143	Collagen Type VII	A non-fibrillar collagen involved in anchoring the epidermal BASEMENT MEMBRANE to underlying tissue. It is a homotrimer comprised of C-terminal and N-terminal globular domains connected by a central triple-helical region.	Collagen Type VII, alpha1 Chain,Collagen Type VII, alpha1 Subunit,Collagen alpha1(VII),Procollagen Type VII,Type VII Collagen,Type VII Procollagen,Collagen, Type VII,Procollagen, Type VII,Type VII, Procollagen
D064987	Cell- and Tissue-Based Therapy	Therapies that involve the TRANSPLANTATION of CELLS or TISSUES developed for the purpose of restoring the function of diseased or dysfunctional cells or tissues.	Cell Therapy,Tissue Therapy,Therapy, Cell,Therapy, Tissue,Cell and Tissue Based Therapy