Sirs, Recessive dystrophic epidermolysis bullosa (RDEB) is characterised by painful mucocutaneous blisters, resulting in oesophageal strictures, mutilating scars, local and systemic infections and syndactyly. RDEB is caused by COL7A1 gene mutations, encoding type VII collagen (1). Antitype VII collagen as well as other anti-skin autoantibodies (anti-BP180, anti-BP230) can be detected, but their pathogenic role is unclear (2). The potential occurrence of AA-amyloidosis suggest that moderate to severe forms of RDEB are systemic inflammatory disorders rather than local skin diseases, thus being eligible for systemic immunomodulating therapy (3).