Treatment of the<unk>  by transplantation of laminin5 modified stem cells Recent research has focused on changing the mixture of keratins produced in the skin. There are 54 known keratin genes—of which 28 belong to the type I intermediate filament genes and 26 to type II—which work as heterodimers. Many of these genes share substantial structural and functional similarity, but they are specialized to cell type and/or conditions under which they are normally produced. If the balance of production could be shifted away from the mutated, dysfunctional keratin gene toward an intact keratin gene, symptoms could be reduced. For example, sulforaphane, a compound found in broccoli, was found to reduce blistering in a mouse model to the point where affected pups could not be identified visually, when injected into pregnant mice (5 μmol/day = 0.9 mg) and applied topically to newborns (1 μmol/day = 0.2 mg in jojoba oil). As of 2008 clinical research at the University of Minnesota has explored allogeneic bone marrow transplantation for RD and junctional<unk> , treating a two-year-old child who is one of two brothers with<unk> . A second transplant has also been performed on the child's older brother, and a third transplant is scheduled for a California baby. A clinical trial is planned for 30 subjects. However, the immune suppression that bone marrow transplantation requires causes a risk of serious infections with large scale blisters and skin erosion. Indeed, at least four people have died in the course of either preparation for or institution of bone marrow transplantation for<unk> , out of only a small group of patients treated so far. The mechanism of action of this therapy is unclear as hematopoietic stem cells are not thought to contribute to epithelial lineages. Rather, it is speculated that cross-correction from tissue-resident graft-derived immune cells contributes to the observed clinical benefit. A pilot study performed in 2015 suggests that systemic granulocyte-colony stimulating factor (G-CSF) may promote increased wound healing in people with dystrophic<unk> . Transplanting skin derived from genetically modified stem cells onto the wound surfaces has been studied with a report of improvements in one person. A 2017 clinical trial with male RDEB (recessive dystrophic<unk>  patients conducted successful grafting of type VII gene corrected keratinocytes (COL7A1 gene correction using retrovirus transduction), without any serious adverse effects. Type VII collage formation was observed at the dermis-epidermis junction in significant amounts. A 2020 study demonstrated the safe allogenic grafting of acellular dermal matrix/scaffolds in<unk>  patients without any observed infection or necrosis and instead noted fewer required dressing changes, promoted wound healing, pain reduction, and an overall improvement in the quality of life of the patients. Monitoring The<unk>  Disease Activity and Scarring index <unk> DASI) is a scoring system that objectively quantifies the severity of<unk> . The<unk> DASI is a tool for clinicians and patients to monitor the severity of the disease. It has also been designed to evaluate the response to new therapies for the treatment of<unk> . The<unk> DASI was developed and validated by Professor Dedee Murrell and her team of students and fellows at the St George Hospital, University of New South Wales, in Sydney, Australia. It was presented at the International Investigative Dermatology congress in Edinburgh in 2013 and a paper-based version was published in the Journal of the American Academy of Dermatology in 2014.
Epidermolysis bullosa
Pemphigus vulgaris
Tinea imbricata
Bullous pemphigoid
Linitis plastica
Diabetic bulla
Ichthyosis bullosa of Siemens
Lichen myxedematosus