Epidermolysis bullosa (EB) comprises a group of heritable skin diseases characterized by fragile skin and mucous membranes and the formation of blisters in response to mechanical trauma or not. EB has a wide spectrum of phenotypes, ranging from mild features due to subtle molecular defects to severe cutaneous and extracutaneous manifestations due to severely compromised dermal-epidermal adhesion. Patients with EB need special care aimed at treating both the primary manifestations of the disease and their potential complications, which, in some cases, can be fatal.

As the areas of the body most often affected are sites subject to frequent pressure or friction, these conditions are also called mechanobullous disorders. The clinical complexity of this, as yet, incurable disease is further increased by extracutaneous manifestations, which include the involvement of skin adnexa, teeth, and the gastrointestinal, urinary tract, and pulmonary epithelia. There is presently no definitive cure for EB and the objective of treatment is to alleviate symptoms and provide supportive measures. Therapy is therefore focused on the prevention of lesions and complications.

Aminoglycosides are a class of antibacterials used mainly to treat Gram-negative bacterial infections Genetic and biochemical studies demonstrate that these antibiotics bind to the decoding site of ribosomal RNA of both and bring about a conformational change that permits codon–anticodon pairing during translation. Aminoglycosides reduce discrimination between cognate and near-cognate tRNA, permitting an amino acid to be inserted at the stop codon. The net effect is continuation of translation through to the natural stop codon expression and because of this it has been used in treatment of cystic fibrosis and ongoing trials on duchenne muscular dystrophy. In a recent study it has been seen to have similar effect in recesive dystrophic EB

In this study we plan to evaluate the efficacy of aminoglycosides in promoting wound healing and prevention of new blister formation in patients of EB. All the patients will be advised to apply topical gentamicin in a collagen base in one half of the body and paraffin guaze dressings on the other side for 12 weeks and the sides are reversed after 12 weeks. Clinical photography, physician global assessment will done monthly to evaluate ulcer healing and biopsy taken to evaluate the expression of proteins before and after treatment on both sides at 0, 12 and 24 weeks.  Paraffin guaze dressings side will act as control. Adequate precaution will be taken to monitor nephro and ototoxicity due to aminoglycosides by doing renal function tests weekly for 1 month and thereafter monthly and audiometry/BERA once every 2 months.