The underlying genetic abnormalities of epidermolysis bullosa (EB) cause destabilization at the dermo-epidermal junction. Patients with EB characteristically are subject to blistering following relatively minor trauma (the Nikolsky sign), and…
Epidermolysis bullosa (EB) results from genetic defects of molecules in the skin concerned with adhesion. Some of the most common problems seen with EB sufferers are blisters, vesicles, and bullas….
Patients with epidermolysis bullosa (EB) may present for anesthesia with an unrelated surgical condition or, more commonly, for diagnostic or therapeutic procedures. Children in particular may require frequent anesthetics. Safe…
Epidermolysis bullosa (EB) is a spectrum of rare, inherited, blistering skin disorders, primarily affecting the skin and pharyngoesophageal mucosa. EB affects approximately 2 to 4 per 100,000 children each year….
Dental treatment is an important part of the multidisciplinary care of patients with epidermolysis bullosa (EB). Routine dental treatment can be difficult to provide in patients with severe tissue fragility…
Optimization of resistance to infection, growth, sexual maturation, wound healing, and provision of the best possible overall quality of life are important management goals in children with epidermolysis bullosa. However,…
Squamous cell carcinomas (SCCs) are highly aggressive in patients with epidermolysis bullosa (EB). Non–ultraviolet-related SCCs are the leading cause of death in patients with recessive dystrophic EB, particularly recessive dystrophic…
Pain is an unfortunate constant in the lives of most patients with epidermolysis bullosa (EB), especially for those with the more severe types of EB. Patients with EB have a…
Tests to monitor in patients with severe types of epidermolysis bullosa are presented. Investigation Comment Chemical Pathology Urea and electrolytes: Na, K, Ur, Cr 6 monthly unless abnormal Liver function…
In all forms of epidermolysis bullosa (EB), skin fragility may result in bacterial colonization or infection, particularly in the more severe forms where wounds may be multiple and long-standing. A…
