Use of nrf2 inducers to treat epidermolysis bullosa simplex and related diseases

Abstract

The present invention relates to methods and compositions for the prevention and treatment of keratin-based skin diseases. In particular, the application describes compositions and methods of treating a patient suffering from skin blistering comprising the use of phase II enzyme inducers.

1 .- 54 . (canceled) 
     
     
         55 . A composition for topical application to the skin comprising a therapeutically effective amount of an Nrf2 inducer and a carrier that allows the Nrf2 inducer to reach basal keratinocytes in the epidermis. 
     
     
         56 . The composition of  claim 55  in the form of ointment, cream, emulsion, lotion or gel. 
     
     
         57 . The composition of  claim 55 , wherein the Nrf2 inducer is a phase II enzyme inducer. 
     
     
         58 . The composition of  claim 57 , wherein the phase II inducer is an isothiocyanate. 
     
     
         59 . The composition of  claim 57 , wherein the phase II enzyme inducer is sulforaphane. 
     
     
         60 . The composition of  claim 57 , wherein the phase II enzyme inducer is a sulforaphane synthetic analogue. 
     
     
         61 . The composition of  claim 60 , wherein the sulforaphane synthetic analogue is exo-2-acetyl-6-isothiocyanatonorbornane. 
     
     
         62 . The composition of  claim 55 , wherein the Nrf2 inducer is keratinocyte growth factor. 
     
     
         63 . The composition of  claim 55 , wherein the Nrf2 inducer is oltipraz. 
     
     
         64 . The composition of  claim 55 , wherein the Nrf2 inducer is ethacrynic acid. 
     
     
         65 . The composition of  claim 55 , wherein the Nrf2 inducer causes the selective induction of K6, K16 or K17 in the keratinocytes in the skin of the patient. 
     
     
         66 . The composition of  claim 55 , wherein the amount of Nrf2 inducer is from about 100 nmol to about 1 μmol/cm 2 . 
     
     
         67 . The composition of  claim 66 , wherein the composition further comprises one or more pharmaceutical, biological or molecular biological active agents.