Atrophoderma of Pasini and Pierini
- Elena Pope, MD, MSc, FRCPC
Elena Pope, MD, MSc, FRCPC
- Professor of Pediatrics
- University of Toronto
- Head, Section of Pediatric Dermatology
- The Hospital for Sick Children, Toronto
- Ronald Laxer, MD, FRCPC
Ronald Laxer, MD, FRCPC
- Professor of Pediatrics
- University of Toronto
Atrophoderma of Pasini and Pierini (APP) is a rare skin disorder affecting dermal collagen and presenting with dermal atrophy. The classic clinical manifestations of APP are hyperpigmented or hypopigmented, depressed areas of skin on the trunk or extremities (picture 1). It is unclear whether some cases of APP result from Borrelia burgdorferi infection. It is also uncertain whether APP is a distinct disease entity or a disorder on the spectrum of morphea (localized scleroderma).
The clinical manifestations, diagnosis, and treatment of APP will be reviewed here. Morphea is reviewed separately. (See "Pathogenesis, clinical manifestations, and diagnosis of morphea (localized scleroderma) in adults" and "Treatment of morphea (localized scleroderma) in adults" and "Localized scleroderma in childhood".)
APP was described in 1923 by Pasini as “atrofodermia idiopathica progressiva”  and later by Pierini as a disorder affecting primarily females and consisting of single or multiple depressed patches with sharp, well-defined borders . In 1958, Canizares coined the term “idiopathic atrophoderma of Pasini and Pierini,” crediting both Italian doctors .
APP is rare. The exact incidence and prevalence are unknown. Most cases have been reported in whites, particularly in European countries. Whether this relates to a higher prevalence of Borrelia infection in Europe or speaks more to easier clinical recognition of this entity in lighter skin individuals is not clear. (See 'Pathogenesis' below.)
APP tends to present [3,4] in the second or third decade of life. However, pediatric onset of the disease has been reported, including congenital presentations [5-8]. APP is seen more frequently in females than in males .
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