Atrophoderma of Pasini and Pierini Clinical Presentation

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

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.

Next

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 legs 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.

Note the images below.

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

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, shiny sclerodermatous indurations are sometimes seen in the central parts of the lesions, and concurrent but separate characteristic plaques of morphea may be noted, supporting a possible relation between these disorders.

Previous
Next

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 Rufi[8] 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.

Previous
Proceed to Differential Diagnoses
 
 
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

  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. 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].

  10. 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].

  11. 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].

  12. 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].

  13. 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].

  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].

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

  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].

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

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

  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].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.