Successful renal transplant in a patient with non-Herlitz junctional epidermolysis bullosa.

Non-Herlitz junctional epidermolysis bullosa (NH-JEB) is a very rare inherited disorder, with an array of complications. We present the case of a 33-year-old patient of Chinese origin, diagnosed with NH-JEB in childhood, who developed severe IgA nephropathy. His renal impairment was initially treated by haemodialysis. He underwent successful renal transplantation, resulting in normalization of his renal function. To our knowledge, this is the first report of renal transplantation in a patient with epidermolysis bullosa, which should support use of this intervention in other similar cases.

The psychosocial impact of chronic wounds on patients with severe epidermolysis bullosa.

OBJECTIVE: To explore the lived experience of individuals with chronic wounds associated with dystrophic and junctional epidermolysis bullosa (EB),to improve understanding and, therefore, enhance the care provided to this group of patients by acquiring in depth data on the psychosocial issues that affect them. METHOD: A phenomenological study using interpretive phenomenological analysis was employed. A purposive sampling method was used with six individuals replying to postal invitation to participate. RESULTS: Following one-to-one interviews, six superordinate themes were identified. These were: coping, pain, perceptions, emotional impact, social impact and support network, each with subordinate themes. All of the superordinate themes have been identified by previous research into chronic wounds, burns and disfiguring conditions; however, new subordinate themes arose. CONCLUSION: This study highlighted the need for individuals with EB to have a multidisciplinary approach to their care with a particular need for pain management, psychological intervention and nursing support from those whom clients perceive as understanding the requirements of patients with EB. Further research into identity issues in individuals with EB is advocated. DECLARATION OF INTEREST: There were no external sources of funding for this study.The authors have no conflicts of interest to declare.