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SEPTEMBER 2016 | The Surgical Technologist | 403 Treating the Epidermolysis Bullosa Patient L E A R N I N G O B J E C T I V E S s Learn about the extreme care needed when dealing with EB patients s Identify the various ailments that are commonly associated with this disease s Review the protocols for room set up and patient prep when preparing for an EB case s List the considerations mentioned for induction and intubation s Recall the procedures for maintaining an airway in EB patients There have been more than 20 types of EB identified; however, they are typically categorized into three groups: EB simplex, dystrophic EB and junction EB. 1 EB occurs roughly 1 in every 20,000 births, with 92% of these cases presenting as EB simplex, 5% as dystrophic EB and 1% as junctional EB. 7 The remaining 2% are unclassified. The main parts of the body affected by EB are the airway, oral cavity, gastrointestinal system, extremities and skin. All types of EB can present with scarring of the soft and mucosal tissue, which may result in strictures of the airway or failure of oral feeding. This is a particular concern for all surgical patients with EB as their airway may be difficult to maintain. Another common defect associated with EB is pseudosyndactyly, or mitten hand deformity. Finally, patients with epidermolysis bullosa are highly susceptible to various forms of skin cancer, especially squamous cell carcinoma. 2 While there is no cure for EB, there are several surgical treatments that can help reduce the crippling effects of the disease as well as Kather i ne Haye s , cst Epidermolysis bullosa (EB) is the name given to a group of congenital genetic disorders resulting in the defect of proteins that promote adhe- sion of the epidermal cells to each other and to the basement membrane. This results in three effects: the first is weakness of the epidermal layer - which is prone to shearing or separation from the basement membrane; the second is bullae or blister formations; and the third is scarring during healing after minor trauma. 6