eMedicine Specialties > Dermatology > Diseases of the Dermis

Atrophoderma of Pasini and Pierini

Author: Anne Laumann, MB, ChB, MRCP(UK), FAAD, Associate Professor, Department of Dermatology, Feinberg School of Medicine, Northwestern University
Coauthor(s): Neelam Vashi, MD, Northwestern University, Feinberg School of Medicine
Contributor Information and Disclosures

Updated: Feb 9, 2009

Introduction

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, Pasini1 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 Vivoli2 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 al4 reported that skin glycosaminoglycans extracted from idiopathic atrophoderma of Pasini and Pierini lesions are different from those in typical morphea lesions.

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. 

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 Borrelia species.

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.

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

Clinical

History

  • Idiopathic atrophoderma of Pasini and Pierini is typically a benign, asymptomatic condition.
  • The disorder usually begins during adolescence or early adulthood with a slightly erythematous lesion appearing on the trunk, commonly on the back or lumbosacral region, followed in frequency by the chest, arms, and abdomen. 
  • At first, only a single lesion may be present, but more often, multiple lesions 1-12 cm in diameter are present.
  • Within 1-2 weeks, these lesions become hyperpigmented, appearing slate-gray to brown.
  • Initially, localized lesions may slowly spread for months to years before becoming quiescent.  Discrete new lesions may appear for 10-20 years, and old lesions may slowly enlarge, giving the skin a moth-eaten appearance.
  • Transformation to generalized morphea has not been observed.

Physical

  • Lesions are single or multiple, irregularly round or ovoid patches, varying in size from a few centimeters to large areas across the trunk. A predilection for the lower extremities is reported.6 The face, hands, and feet are usually spared. Distribution is often symmetric and bilateral; however, reports have described solely unilateral cases.7 Lesions have traditionally been described as hyperpigmented; however, Saleh et al described a retrospective study of 16 Lebanese patients in whom lesions were more hypopigmented (56%) and skin-colored (25%).6
  • The lesions are usually asymptomatic and lack inflammation.
  • The skin lesions may coalesce over time to form large, irregular, pigmented areas. The areas usually are brown but may have a blue hue.
  • Eventually, the pigmentation lightens, and the involved skin becomes depressed below the level of the surrounding skin. This change produces the characteristic, sharply defined "cliff-drop" borders, ranging from 1-8 mm in depth, although they can have a gradual slant. Close examination of the skin with side lighting demonstrates the unique cliff-drop border, giving the impression of an inverted plateau. Multiple lesions may have an appearance similar to Swiss cheese. 
  • The skin surrounding the patches appears normal. No erythema or lilac ring, as in morphea, is observed.
  • Once indentation occurs, the lesions may become quiescent, stopping any further enlargement.
  • The surface of the skin feels and appears normal, and no induration or sclerosis is noted.
  • In the late stage, superficial blood vessels may be visible in the depressed patches.  No palpable difference can be felt between normal and affected skin in the late stage.
  • White indurations are sometimes seen in the central parts of the lesions, and concurrent but separate characteristic plaques of morphea may be noted.

Causes

  • Idiopathic atrophoderma of Pasini and Pierini may represent a late atrophic stage of morphea.
  • Some findings suggest that the spirochete B burgdorferi may be involved in the pathogenesis of some cases of idiopathic atrophoderma of Pasini and Pierini.
  • Buechner and Rufi8  studied the sera of 26 patients with typical lesions. Ten (53%) of the 26 patients had immunoglobulin G anti– B burgdorferi antibodies, and 6 (14%) of control subjects had these antibodies. No immunoglobulin M antibodies were found.

More on Atrophoderma of Pasini and Pierini

Overview: Atrophoderma of Pasini and Pierini
Differential Diagnoses & Workup: Atrophoderma of Pasini and Pierini
Treatment & Medication: Atrophoderma of Pasini and Pierini
Follow-up: Atrophoderma of Pasini and Pierini
Multimedia: Atrophoderma of Pasini and Pierini
References
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References

  1. Pasini A. Atrofodermia idiopatica progressiva. G Ital Dermatol. 1923;58:785.

  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].

  6. Saleh Z, Abbas O, Dahdah MJ, Kibbi AG, Zaynoun S, Ghosn S. Atrophoderma of Pasini and Pierini: a clinical and histopathological study. J Cutan Pathol. Dec 2008;35(12):1108-14. [Medline].

  7. Miteva L, Kadurina M. Unilateral idiopathic atrophoderma of Pasini and Pierini. Int J Dermatol. Nov 2006;45(11):1391-3. [Medline].

  8. 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. Mar 1994;30(3):441-6. [Medline].

  9. Abe I, Ochiai T, Kawamura A, Muto R, Hirano Y, Ogawa M. Progressive idiopathic atrophoderma of Pasini and Pierini: the evaluation of cutaneous atrophy by 13-MHz B-mode ultrasound scanning method. Clin Exp Dermatol. May 2006;31(3):462-4. [Medline].

  10. Kernohan NM, Stankler L, Sewell HF. Atrophoderma of Pasini and Pierini. An immunopathologic case study. Am J Clin Pathol. Jan 1992;97(1):63-8. [Medline].

  11. Carter JD, Valeriano J, Vasey FB. Hydroxychloroquine as a treatment for atrophoderma of Pasini and Pierini. Int J Dermatol. Oct 2006;45(10):1255-6. [Medline].

  12. Arpey CJ, Patel DS, Stone MS, Qiang-Shao J, Moore KC. 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(3):206-12. [Medline].

  13. 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].

  14. 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].

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

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

  17. 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].

  18. 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].

  19. Heymann WR. Coexistent lichen sclerosus et atrophicus and atrophoderma of Pasini and Pierini. Int J Dermatol. Feb 1994;33(2):133-4. [Medline].

  20. 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].

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

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

  23. 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.

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

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

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

  27. Stainislaw AB, Theo R. Atrophoderma of Pasini and Pierini. J Am Acad Dermatol. 1994;30:441-6.

  28. Wakelin SH, James MP. Zosteriform atrophoderma of Pasini and Pierini. Clin Exp Dermatol. May 1995;20(3):244-6. [Medline].

  29. 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].

  30. 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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Further Reading

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Keywords

idiopathic atrophoderma of Pasini and Pierini, IAPP, atrophodermia idiopathica progressiva, progressive idiopathic atrophoderma, scleroderma, morphea, lichen sclerosus et atrophicus, B burgdorferi, Borrelia burgdorferi

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Contributor Information and Disclosures

Author

Anne Laumann, MB, ChB, MRCP(UK), FAAD, Associate Professor, Department of Dermatology, Feinberg School of Medicine, Northwestern University
Anne Laumann, MB, ChB, MRCP(UK), FAAD is a member of the following medical societies: American Academy of Dermatology, Association of Professors of Dermatology, 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: Nothing to disclose.

Coauthor(s)

Neelam Vashi, MD, Northwestern University, Feinberg School of Medicine
Neelam Vashi, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Physicians of Indian Origin, American Medical Association, American Medical Student Association/Foundation, and Indian American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

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
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.

Managing Editor

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.

CME Editor

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, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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