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Idiopathic atrophoderma of Pasini and Pierini (IAPP) is a rare, exclusively cutaneous condition which occurs most frequently in female patients, with a peak incidence in the second and third decades of life. Overall, fewer than 100 cases have been reported in the literature. The pathophysiology of this entity remains unknown and the role of Borrelia burgdorferi infections remains controversial. Some researchers believe that IAPP should be included as a manifestation of the spectrum of localised scleroderma. Some cases of familial IAPP have been described; however, a genetic link has not yet been confirmed.