The Conundrum of Allogeneic Bone Marrow Transplantation for Epidermolysis Bullosa  Jouni Uitto  Journal of Investigative Dermatology  Volume 138, Issue 5, Pages 1029-1031 (May 2018) DOI: 10.1016/j.jid.2017.12.009 Copyright © 2017 The Author Terms and Conditions

Figure 1 Conceptual illustration of bone marrow transplantation (BMT) for the treatment of epidermolysis bullosa (EB). BMT results in chimeric engraftment of donor-derived wild-type cells, including PDGFRα+/CXCR4+ mesenchymal stem cells (MSCs), into the recipient’s bone marrow. Necrotic skin in patients with EB releases cytokines and signaling molecules, such as high-mobility group box 1 (HMGB1) that recruits bone marrow-derived MSCs to circulation and subsequently homing to damaged skin through the stromal cell-derived factor-1α (SDF-1α)/CXCR4 axis. The damaged skin microenvironment creates a niche conducive for transdifferentiation of MSCs into keratinocytes (KCs) and fibroblasts (FBs) that elicit synthesis of proteins deficient in EB leading to tissue regeneration counteracting the disease. (Adapted from Tamai and Uitto, 2016.) Journal of Investigative Dermatology 2018 138, 1029-1031DOI: (10.1016/j.jid.2017.12.009) Copyright © 2017 The Author Terms and Conditions