We describe two siblings with late-onset junctional epidermolysis bullosa (JEB) (formerly called epidermolysis junctionalis progressiva). This is a subtype of autosomal recessive JEB characterized by late onset of the symptoms, between the ages of 5 and 8 years. The symptoms are mechanobullous lesions preferentially situated on hands and feet, nail dystrophy, loss of dermatoglyphic pattern, tooth enamel abnormalities and hyperhidrosis. In most forms of JEB a reduction or absence of a specific hemidesmosomal component can be demonstrated by means of immunohistochemical analysis. In this family, all known involved hemidesmosomal components, including uncein, recognized by the monoclonal antibody 19-DEJ-1, appeared to be normally expressed.