Atrophoderma of Pasini and Pierini 

  • Author: Anne Elizabeth Laumann, MBChB, MRCP(UK), FAAD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 12, 2011
 

Background

Idiopathic atrophoderma of Pasini and Pierini (IAPP) is a form of dermal atrophy that manifests as single or multiple sharply demarcated, hyperpigmented, nonindurated patches. These patches are marked by a slight depression of the skin with an abrupt edge, usually on the backs of adolescents or young adults. The lesions may be discrete or confluent. The affected skin appears thinned and discolored, but the consistency and feel of the affected skin remains normal.

In 1923, Pasini[1] described a case of pigmentary atrophoderma that was both clinically and histologically unique from any known atrophy, including localized scleroderma, under the name progressive idiopathic atrophoderma. In 1936, in Argentina, Pierini and Vivoli[2] extensively studied and defined the condition and its possible link to morphea. In 1958, the disorder was first introduced into the American dermatologic literature by Canizares et al,[3] who reviewed Pierini’s findings and renamed it idiopathic atrophoderma of Pasini and Pierini. Canizares et al believed that idiopathic atrophoderma of Pasini and Pierini differed sufficiently from morphea to classify it as a distinct entity.

Since then, reports of the co-occurrence of morphea and occasionally lichen sclerosus et atrophicus with idiopathic atrophoderma of Pasini and Pierini suggest a close relationship between idiopathic atrophoderma of Pasini and Pierini and morphea. In 2000, Yokoyama et al[4] reported that skin glycosaminoglycans extracted from idiopathic atrophoderma of Pasini and Pierini lesions are different from those in typical morphea lesions.

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Pathophysiology

The cause of atrophoderma of Pasini and Pierini is not known. The pathophysiologic events that cause the discrete lesions seen clinically, as well as the timing of their appearance, are also unknown. Some authors have suggested a role for infection with Borrelia burgdorferi.

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Epidemiology

Frequency

United States

The exact incidence of idiopathic atrophoderma of Pasini and Pierini is not known. The small number of cases reported may reflect its asymptomatic nature rather than its true incidence.

International

Idiopathic atrophoderma of Pasini and Pierini is more frequently reported in Europe than in North America, paralleling the incidence of acrodermatitis chronica atrophicans and suggesting involvement of European Borrelia strains.

Mortality/Morbidity

Idiopathic atrophoderma of Pasini and Pierini is a benign, asymptomatic disease and is not associated with any significant complications or mortality.

Race

Idiopathic atrophoderma of Pasini and Pierini is more common in whites and is rarely reported in blacks or Asians.

Sex

Idiopathic atrophoderma of Pasini and Pierini is more frequently encountered in women than in men, with a ratio of 6:1.

Age

Idiopathic atrophoderma of Pasini and Pierini usually begins insidiously in individuals during the second or third decade of life. However, it has been described in individuals as young as 7 years and as old as 66 years, with one report of congenital atrophoderma.[5]

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Contributor Information and Disclosures
Author

Anne Elizabeth Laumann, MBChB, MRCP(UK), FAAD  Associate Professor, Department of Dermatology, Northwestern University, The Feinberg School of Medicine

Anne Elizabeth Laumann, MBChB, MRCP(UK), FAAD is a member of the following medical societies: American Academy of Dermatology, Association of Professors of Dermatology, British Association of Dermatologists, Chicago Dermatological Society, Chicago Medical Society, Illinois Dermatological Society, Illinois State Medical Society, Illinois State Medical Society, Medical Dermatology Society, and Society for Investigative Dermatology

Disclosure: Abbott Grant/research funds Other; Regeneron Grant/research funds Other; Centocor Grant/research funds Other; OSI Grant/research funds Other; Celgene Grant/research funds Other; Lilly Grant/research funds Other

Coauthor(s)

Neelam Vashi, MD  Resident Physician, Department of Dermatology, New York University Medical Center

Neelam Vashi, MD is a member of the following medical societies: American Academy of Dermatology, American Association of Physicians of Indian Origin, American Medical Association, and Indian American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Peter Fritsch, MD  Chair, Department of Dermatology and Venereology, University of Innsbruck, Austria

Peter Fritsch, MD is a member of the following medical societies: American Dermatological Association, International Society of Pediatric Dermatology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Lester F Libow, MD  Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Texas Medical Association

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgments

We gratefully acknowledge the contributions of Dr. Richard H. Musgnug, author of the previous edition of this article.

References

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  2. Pierini L, Vivoli D. Atrofodermia progressiva (Pasini). G Ital Dermatol. 1936;77:403-09.

  3. Canizares O, Sachs PM, Jaimovich L, Torres VM. Idiopathic atrophoderma of Pasini and Pierini. AMA Arch Derm. Jan 1958;77(1):42-58; discussion 58-60. [Medline].

  4. Yokoyama Y, Akimoto S, Ishikawa O. Disaccharide analysis of skin glycosaminoglycans in atrophoderma of Pasini and Pierini. Clin Exp Dermatol. Jul 2000;25(5):436-40. [Medline].

  5. Kim SK, Rhee Sh, Kim YC, Lee ES, Kang HY. Congenital atrophoderma of Pasini and Pierini. J Korean Med Sci. Feb 2006;21(1):169-71. [Medline].

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  9. Kopec-Medrek M, Kotulska A, Zycinska-Debska E, Widuchowska M, Kucharz EJ. Exacerbated course of atrophoderma of Pasini and Pierini in patient with papillary cancer of the thyroid gland. Wiad Lek. 2010;63(1):24-6. [Medline].

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  14. Aberer E, Kollegger H, Kristoferitsch W, Stanek G. Neuroborreliosis in morphea and lichen sclerosus et atrophicus. J Am Acad Dermatol. Nov 1988;19(5 Pt 1):820-5. [Medline].

  15. Berman A, Berman GD, Winkelmann RK. Atrophoderma (Pasini-Pierini). Findings on direct immunofluorescent, monoclonal antibody, and ultrastructural studies. Int J Dermatol. Sep 1988;27(7):487-90. [Medline].

  16. Browne C, Fisher BK. Atrophoderma of moulin with preceding inflammation. Int J Dermatol. Nov 2000;39(11):850-2.

  17. Buechner SA, Rufli T. Progressive Idiopathic Atrophoderma of Pasini and Pierini-a Borrelia disease. Zentralbl Bakt Med Microbiol Hyg Suppl. 1989;18:1164-71.

  18. Buechner SA, Winkelmann RK, Lautenschlager S, Gilli L, Rufli T. Localized scleroderma associated with Borrelia burgdorferi infection. Clinical, histologic, and immunohistochemical observations. J Am Acad Dermatol. Aug 1993;29(2 Pt 1):190-6. [Medline].

  19. Franck JM, MacFarlane D, Silvers DN, Katz BE, Newhouse J. Atrophoderma of Pasini and Pierini: atrophy of dermis or subcutis?. J Am Acad Dermatol. Jan 1995;32(1):122-3. [Medline].

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  21. Jablonska S, Blaszczyk M. Is superficial morphea synonymous with atrophoderma Pasini-Pierini?. J Am Acad Dermatol. Jun 2004;50(6):979-80; author reply 980. [Medline].

  22. Jeanselme E, Burnier R. Sclerodermie en plaques avec dyschromie pigmentaire symmetrique. Bull Soc Fr Dermatol Syph. 1926;33:704-06.

  23. Kencka D, Blaszczyk M, Jablonska S. Atrophoderma Pasini-Pierini is a primary atrophic abortive morphea. Dermatology. 1995;190(3):203-6. [Medline].

  24. Murphy PK. Concomitant unilateral idiopathic atrophoderma of Pasini and Pierini and morphea (case report of IAPP in a black man). Int J Dermatol. 1940;29:281-3.

  25. Per M. Ein Fall von Sclerodermie superficiallis circumscripta en plaques. Venerol (Russ). 1926;4:578-83.

  26. Per M. Oberflachliche, circumscripta Sclerodermie. Handbuch. 1931;8:893.

  27. Pullara TJ, Lober CW, Fenske NA. Idiopathic atrophoderma of Pasini and Pierini. Int J Dermatol. Dec 1984;23(10):643-5. [Medline].

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  30. Wielowieyska-Szybinska D, Wojas-Pelc A, Dyduch G, Zawisz A. [Type I and II collagens and mast cells expression in the skin lesions from the patients with localized scleroderma]. Przegl Lek. 2008;65(4):161-5. [Medline].

  31. Wojas-Pelc A, Wielowieyska-Szybinska D, Kieltyka A. [Presence of the antinuclear antibodies and antibodies to Borrelia burgdorferi among patients with morphea en plaque, deep linear scleroderma and atrophoderma Pasini-Pierini]. Przegl Lek. 2002;59(11):898-902. [Medline].

 
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Early lesion demonstrating diagnostic "cliff-drop" border to atrophy. Courtesy of Joe Eastern, MD.
Older lesion showing typical pigmentation and classic "cliff-drop" border. Courtesy of Joe Eastern, MD.
 
 
 
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