Idiopathic Atrophoderma of Pasini and Pierini

Background: Idiopathic atrophoderma of Pasini and Pierini (LAPP) is a rare pattern of dermal atrophy, with less than 300 cases reported worldwide. Currently, there is controversy surrounding the classification of LAPP; some consider it to be a variant of morphea, while others view it as a separate disease entity. Objective: Our purpose was to further elucidate the nature and course of this unusual disease. Methods: We present a case report, with long-term follow-up of a case of IAPP of 30 years' duration. Results: The patient's lesions were primarily atrophic, without progression to morphea, scleroderma, or other systemic disease. Biopsy of a long-standing plaque demonstrated dermal thinning, and minimal dermal infiltrate. Conclusion: This case supports the findings of the literature on this subject: the majority of patients with IAPP have an entirely benign course, without progression to other disease.