scholarly journals High Local Concentrations of Intradermal MSCs Restore Skin Integrity and Facilitate Wound Healing in Dystrophic Epidermolysis Bullosa

2015 ◽  
Vol 23 (8) ◽  
pp. 1368-1379 ◽  
Author(s):  
Tobias Kühl ◽  
Markus Mezger ◽  
Ingrid Hausser ◽  
Rupert Handgretinger ◽  
Leena Bruckner-Tuderman ◽  
...  
Keyword(s):  
Wound Healing ◽  
Epidermolysis Bullosa ◽  
Dystrophic Epidermolysis Bullosa ◽  
Skin Integrity

scholarly journals Impact of low-dose calcipotriol ointment on wound healing, pruritus and pain in patients with dystrophic epidermolysis bullosa: A randomized, double-blind, placebo-controlled trial

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Christina Guttmann-Gruber ◽  
Josefina Piñón Hofbauer ◽  
Birgit Tockner ◽  
Victoria Reichl ◽  
Alfred Klausegger ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Wound Healing ◽  
Vitamin D3 ◽  
Epidermolysis Bullosa ◽  
Low Dose ◽  
Minimum Size ◽  
Minor Trauma ◽  
Double Blind ◽  
Dystrophic Epidermolysis Bullosa ◽  
Search Trial

Abstract Background Wound management is a critical factor when treating patients with the inherited skin fragility disease dystrophic epidermolysis bullosa (DEB). Due to genetic defects in structural proteins, skin and mucous epithelia are prone to blistering and chronic wounding upon minor trauma. Furthermore, these wounds are commonly associated with excessive pruritus and predispose to the development of life-threatening squamous cell carcinomas, underscoring the unmet need for new therapeutic options to improve wound healing in this patient cohort. Vitamin D3 is acknowledged to play an important role in wound healing by modulating different cellular processes that impact epidermal homeostasis and immune responses. In this study, we evaluate the safety and efficacy of low-dose calcipotriol, a vitamin D3 analogue, in promoting wound healing and reducing itch and pain in patients with DEB. Methods Eligible DEB patients, aged ≥ 6 years and with a known mutation in the COL7A1 gene, were recruited to a placebo-controlled, randomized, double blind, cross-over phase II monocentric clinical trial. Patients were required to have at least two wounds with a minimum size of 6 cm2 per wound. The primary objective was to evaluate efficacy of daily topical application of a 0.05 µg/g calcipotriol ointment in reducing wound size within a 4-week treatment regimen. Secondary objectives were to assess safety, as well as the impact of treatment on pruritus, pain, and bacterial wound colonization in these patients. Results Six patients completed the clinical trial and were included into the final analysis. Topical low-dose calcipotriol treatment led to a significant reduction in wound area at day 14 compared to placebo (88.4% vs. 65.5%, P < 0.05). Patients also reported a significant reduction of pruritus with calcipotriol ointment compared to placebo over the entire course of the treatment as shown by itch scores of 3.16 vs 4.83 (P < 0.05) and 1.83 vs 5.52 (P < 0.0001) at days 14 and 28, respectively. Treatment with low-dose calcipotriol did not affect serum calcium levels and improved the species richness of the wound microbiome, albeit with no statistical significance. Conclusions Our results show that topical treatment with low-dose calcipotriol can accelerate wound closure and significantly reduces itch, and can be considered a safe and readily-available option to improve local wound care in DEB patients. TrialRegistration EudraCT: 2016–001,967-35. Registered 28 June 2016, https://www.clinicaltrialsregister.eu/ctr-search/trial/2016-001967-35/AT

Cultured keratinocyte allografts and wound healing in severe recessive dystrophic epidermolysis bullosa

1993 ◽  
Vol 29 (3) ◽  
pp. 407-419 ◽  
Author(s):  
John A. McGrath ◽  
Olivia M.V. Schofield ◽  
Akemi Ishida-Yamamoto ◽  
Anthony O’Grady ◽  
Bryan J. Mayou ◽  
...  
Keyword(s):  
Wound Healing ◽  
Epidermolysis Bullosa ◽  
Dystrophic Epidermolysis Bullosa ◽  
Recessive Dystrophic Epidermolysis Bullosa

scholarly journals Increased abundance of Cbl E3 ligases alters PDGFR signaling in recessive dystrophic epidermolysis bullosa

2021 ◽  
Author(s):  
Esther Martinez Martinez ◽  
Regine Toelle ◽  
Julia Donauer ◽  
Christine Gretzmeier ◽  
Leena Bruckner-Tuderman ◽  
...  
Keyword(s):  
Wound Healing ◽  
Growth Factor ◽  
Signaling Pathways ◽  
Epidermolysis Bullosa ◽  
Tgfβ Signaling ◽  
Dystrophic Epidermolysis Bullosa ◽  
Impaired Wound Healing ◽  
E3 Ligases ◽  
Recessive Dystrophic Epidermolysis Bullosa ◽  
Collagen Vii

In recessive dystrophic epidermolysis bullosa (RDEB), loss of collagen VII, the main component of anchoring fibrils critical for epidermal-dermal cohesion, affects several intracellular signaling pathways and leads to impaired wound healing and fibrosis. In skin fibroblasts, wound healing is also affected by platelet-derived growth factor receptor (PDGFR) signaling. To study a potential effect of loss of collagen VII on PDGFR signaling we performed unbiased disease phosphoproteomics. Whereas RDEB fibroblasts exhibited an overall weaker response to PDGF, Cbl E3 ubiquitin ligases, negative regulators of growth factor signaling, were stronger phosphorylated. This increase in phosphorylation was linked to higher Cbl protein levels due to increased TGFβ signaling in RDEB. In turn, increased Cbl levels led to increased PDGFR ubiquitination, internalization, and degradation negatively affecting MAPK and AKT downstream signaling pathways. Thus, our results indicate that elevated TGFβ signaling leads to an attenuated response to growth factors, which contribute to impaired dermal wound healing in RDEB.

scholarly journals Aberrant recruitment of leukocytes defines poor wound healing in patients with recessive dystrophic epidermolysis bullosa

2020 ◽  
Vol 100 (3) ◽  
pp. 209-216
Author(s):  
Taylor Phillips ◽  
Leonie Huitema ◽  
Rodrigo Cepeda ◽  
Diego de los Cobos ◽  
Regina Isabella Matus Perez ◽  
...  
Keyword(s):  
Wound Healing ◽  
Epidermolysis Bullosa ◽  
Dystrophic Epidermolysis Bullosa ◽  
Recessive Dystrophic Epidermolysis Bullosa

Pseudosyndactyly - an inflammatory and fibrotic wound healing disorder in recessive dystrophic epidermolysis bullosa

2015 ◽  
Vol 13 (12) ◽  
pp. 1257-1266
Author(s):  
Jenny Breitenbach ◽  
Christina Gruber ◽  
Alfred Klausegger ◽  
Andrea Trost ◽  
Barbara Bogner ◽  
...  
Keyword(s):  
Wound Healing ◽  
Epidermolysis Bullosa ◽  
Dystrophic Epidermolysis Bullosa ◽  
Recessive Dystrophic Epidermolysis Bullosa
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