Pustular lesions of palms and soles (pustulosis palmoplantaris – PPP) are a chronic condition with an unspecified nosological position. Some researchers consider it as a variant of psoriasis, whereas others indicate numerous distinctions and consider PPP as a separate entity. Genetic investigations support exclusion of PPP from the psoriasis group. Among concepts of PPP aetiopathogenesis, the one concerning the role of sweating and the theory about PPP as a cutaneous manifestation of a generalized autoimmunological process demand attention. A typical patient is a woman 50-60 years old, a smoker, suffering from thyroid disease. Among other disorders associated with PPP disturbed calcium homeostasis and presence of anti-gliadin antibodies are observed. Among numerous systemic and topical therapies retinoids present the highest, but not satisfactory effectiveness. Attempts to use biological treatments are made as well, but due to the insufficient number of references it is impossible to draw conclusions about their effectiveness. An interesting issue is onset or exacerbation of pustulosis palmoplantaris type of lesions or psoriasis induced by TNF-α antagonist therapy. Further studies on aetiopathogenesis and treatment of PPP are necessary.