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 .To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Pasini A. Atrofodermia idiopatica progressiva. G Ital Dermatol 1923; 58:75.
- Pierini L, Vivoli D. Atrofodermia progressiva (Pasini). G Ital Dermatol 1923; 77:403.
- CANIZARES O, SACHS PM, JAIMOVICH L, TORRES VM. Idiopathic atrophoderma of Pasini and Pierini. AMA Arch Derm 1958; 77:42.
- Pullara TJ, Lober CW, Fenske NA. Idiopathic atrophoderma of Pasini and Pierini. Int J Dermatol 1984; 23:643.
- Kang CY, Lam J. Congenital idiopathic atrophoderma of Pasini and Pierini. Int J Dermatol 2015; 54:e44.
- Kim SK, Rhee SH, Kim YC, et al. Congenital atrophoderma of Pasini and Pierini. J Korean Med Sci 2006; 21:169.
- Handler MZ, Alshaiji JM, Shiman MI, et al. Congenital idiopathic atrophoderma of Pasini and Pierini. Dermatol Online J 2012; 18:4.
- Bassi A, Remaschi G, Difonzo EM, et al. Idiopathic congenital atrophoderma of Pasini and Pierini. Arch Dis Child 2015; 100:1184.
- Buechner SA, Rufli T. Atrophoderma of Pasini and Pierini. Clinical and histopathologic findings and antibodies to Borrelia burgdorferi in thirty-four patients. J Am Acad Dermatol 1994; 30:441.
- Berman A, Berman GD, Winkelmann RK. Atrophoderma (Pasini-Pierini). Findings on direct immunofluorescent, monoclonal antibody, and ultrastructural studies. Int J Dermatol 1988; 27:487.
- Kernohan NM, Stankler L, Sewell HF. Atrophoderma of Pasini and Pierini. An immunopathologic case study. Am J Clin Pathol 1992; 97:63.
- Lee Y, Oh Y, Ahn SY, et al. A Case of Atrophoderma of Pasini and Pierini Associated with Borrelia burgdorferi Infection Successfully Treated with Oral Doxycycline. Ann Dermatol 2011; 23:352.
- Jablonska S, Blaszczyk M. Is superficial morphea synonymous with atrophoderma Pasini-Pierini? J Am Acad Dermatol 2004; 50:979.
- Kencka D, Blaszczyk M, Jabłońska S. Atrophoderma Pasini-Pierini is a primary atrophic abortive morphea. Dermatology 1995; 190:203.
- Saleh Z, Abbas O, Dahdah MJ, et al. Atrophoderma of Pasini and Pierini: a clinical and histopathological study. J Cutan Pathol 2008; 35:1108.
- Avancini J, Valente NY, Romiti R. Generalized lenticular atrophoderma of Pasini and Pierini. Pediatr Dermatol 2015; 32:389.
- Miteva L, Kadurina M. Unilateral idiopathic atrophoderma of Pasini and Pierini. Int J Dermatol 2006; 45:1391.
- Abe I, Ochiai T, Kawamura A, et al. Progressive idiopathic atrophoderma of Pasini and Pierini: the evaluation of cutaneous atrophy by 13-MHz B-mode ultrasound scanning method. Clin Exp Dermatol 2006; 31:462.
- Tan SK, Tay YK. Linear atrophoderma of Moulin. JAAD Case Rep 2016; 2:10.
- Carter JD, Valeriano J, Vasey FB. Hydroxychloroquine as a treatment for atrophoderma of Pasini and Pierini. Int J Dermatol 2006; 45:1255.
- Salsberg JM, Weinstein M, Shear N, et al. Impact of Cosmetic Camouflage on the Quality of Life of Children With Skin Disease and Their Families. J Cutan Med Surg 2016; 20:211.
- Arpey CJ, Patel DS, Stone MS, et al. Treatment of atrophoderma of Pasini and Pierini-associated hyperpigmentation with the Q-switched alexandrite laser: a clinical, histologic, and ultrastructural appraisal. Lasers Surg Med 2000; 27:206.