The case of a 60-year-old man with acral persistent papular mucinosis (APPM), thought to represent a new distinctive form of dermal mucinosis not associated with systemic diseases, is reported. The patient had a 4-year history of multiple small papular lesions on the distal forearms, wrists and back of the hands. Histologically, mucin deposits in the upper and mid dermis sparing a superficial subepidermal grenz zone were observed. In contrast to previously described cases, a monoclonal IgA of kappa light chain isotype was detected. Our findings challenge the view that absence of paraproteinemia is a peculiar characteristic of APPM and raise once more the question of its relationship to the discrete papular form of lichen myxedematosus.