Paciente con Epidermólisis Bullosa Distrófica Recesiva (EB) / Patient with Recessive Dystrophic Epidermolysis Bullosa

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Iterative codesign and testing of a novel dressing glove for epidermolysis bullosa.

OBJECTIVE: Recessive dystrophic epidermolysis bullosa (RDEB) is a rare genetic skin disorder which requires intensive hand therapy to delay fusion of the digits. Existing dressings do not conform to the complex structure of the hand and are applied in patches held with additional bandages, leading to an occlusive environment. The aim was to co-design with patients a dressing glove based on their user experiences and needs. METHOD: Qualitative interviews and focus groups with children and adults with RDEB, and their carers, were conducted. Iterative feedback of design cues, bench and surrogate testing of materials and prototype refinement were achieved through collaborative codesign with patients, carers, clinicians and manufacturers. RESULTS: Thematic analysis generated eight user needs and corresponding design cues, addressing issues of absorbency, adherence, comfort, adaptability, ease of application and removal, breathability, protection, and hand hygiene. A prototype was selected for proof of concept testing. CONCLUSION: This novel dressing glove design met the patient's requirements for a dressing, which conformed to the hand structure and sat in the web spaces to keep fingers separated. Proof of concept testing has since been undertaken with patients to determine performance, value for money and whether further developments are required.

Epidermolysis bullosa in three Chinese neonates.

Epidermolysis bullosa (EB) is one of the rare blistering diseases that may present in the neonatal period. Diagnosis is based on clinical symptomatology, histopathology, electron microscopy and genetic studies. Age of onset, symptomatology and prognosis of the various subtypes are varied. We describe three cases of EB dystrophica (EBD) followed at the paediatric dermatology clinic. All of them were symptomatic and diagnosed in the neonatal period. One patient had very severe disease and was confirmed to have the Hallopeau-Siemens subtype with both copies of his entire chromosome three from his mother. This extremely rare mode of inheritance is known as uniparental isodisomy. The clinical course of the other two neonates was milder and suggested that they had the dominant form of EBD. Many issues associated with the disease may be encountered. Specifically, details in skin care, counselling and advice were provided and reinforced to the parents. A multidisciplinary approach with inputs from the neonatologists, paediatricians, dermatologists, plastic surgeons, dietitians, physiotherapists, occupational therapists, and special nurses are required to assure optimal outcome.

Epidermolysis bullosa: pathophysiology and nursing care.

Epidermolysis bullosa (EB) is defined as a group of skin disorders that presents as blistering of the skin in varying degrees of severity. Most cases are inherited, but rare cases may be acquired. There are three main types: simplex, junctional, and dystrophic. The dermal-epidermal junction of the newborn skin is a vital area of attachment. Any defects in the components that comprise this junction can lead to fragility of the skin. EB diagnosis and testing can be per formed both prenatally and postnatally. There is no cure for EB. Treatment revolves around supportive care and prevention of complications. The NICU nurse plays an important role on the multidisciplinary team as primary caregiver to the infant and educator to the family.