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Atrophoderma of Pasini and Pierini Clinical Presentation

  • Author: Sarah Jane Adams, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Apr 11, 2016
 

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.[9]

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.

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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 has also been reported.[10] The face, hands, and feet are usually spared. Distribution is often symmetric and bilateral; however, reports have described solely unilateral or zosteriform distribution.[11] A rare, generalized, and lenticular variant has also been documented.[9] 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%).[10]

The lesions are usually asymptomatic and lack inflammation. The skin lesions may coalesce over time to form large, irregular, pigmented areas. The areas are usually 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.

Single ovoid patch of atrophoderma on the back of Single ovoid patch of atrophoderma on the back of a young adult female.
Atrophic hyperpigmented patch with characteristic Atrophic hyperpigmented patch with characteristic “cliff-drop” borders.

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 stages, superficial blood vessels may be visible in the depressed patches. No palpable difference can be felt between normal and affected skin. White, shiny sclerodermatous induration is sometimes seen in the central part of the lesions, and concurrent but separate characteristic plaques of morphea may be noted, supporting a possible relationship between these disorders.

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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[12] 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.

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Contributor Information and Disclosures
Author

Sarah Jane Adams, MD Resident Physician, Department of Dermatology, Northwestern University, The Feinberg School of Medicine

Sarah Jane Adams, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Womens Association, Chicago Dermatological Society, Society for Melanoma Research

Disclosure: Nothing to disclose.

Coauthor(s)

Anne Elizabeth Laumann, MBChB, MRCP(UK), FAAD Professor of Dermatology, Chief of General Dermatology, Director of the Collagen Vascular Disorders Clinic, 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, Medical Dermatology Society, Society for Investigative Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

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

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

Pranathi Lingam, MD Resident Physician, Department of Dermatology, University of Michigan Medical School

Disclosure: Nothing to disclose.

Acknowledgements

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.

Richard H Musgnug, MD Former Assistant Clinical Professor, Department of Dermatology, Thomas Jefferson Medical School, Virtua Memorial Hospital, Cooper Medical Center

Disclosure: Nothing to disclose.

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.

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. 1958 Jan. 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. 2000 Jul. 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. Handler MZ, Alshaiji JM, Shiman MI, Elgart GW, Schachner LA. Congenital idiopathic atrophoderma of Pasini and Pierini. Dermatol Online J. 2012 Apr 15. 18(4):4. [Medline].

  7. Kang CY, Lam J. Congenital idiopathic atrophoderma of Pasini and Pierini. Int J Dermatol. 2015 Jan. 54 (1):e44-6. [Medline]. [Full Text].

  8. Bassi A, Remaschi G, Difonzo EM, Greco A, Buccoliero AM, Giani T, et al. Idiopathic congenital atrophoderma of Pasini and Pierini. Arch Dis Child. 2015 Dec. 100 (12):1184. [Medline]. [Full Text].

  9. Avancini J, Valente NY, Romiti R. Generalized lenticular atrophoderma of Pasini and Pierini. Pediatr Dermatol. 2015 May-Jun. 32 (3):389-91. [Medline].

  10. 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. 2008 Dec. 35(12):1108-14. [Medline].

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

  12. 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 Mar. 30 (3):441-6. [Medline].

  13. Lis-Święty A, Bierzyńska-Macyszyn G, Arasiewicz H, Brzezińska-Wcisło L. Bilateral atrophoderma linearis: a relationship between atrophoderma linearis Moulin and atrophoderma Pasini-Pierini?. Int J Dermatol. 2016 Mar. 55 (3):339-41. [Medline]. [Full Text].

  14. Batista CM, Lemos MO, Franceschi LE, Basilio CB, Reis CM. Case for diagnosis. An Bras Dermatol. 2014 Jul-Aug. 89 (4):671-3. [Medline].

  15. 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].

  16. 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. 2006 May. 31(3):462-4. [Medline].

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

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

  19. Lee Y, Oh Y, Ahn SY, Park HY, Choi EH. A Case of Atrophoderma of Pasini and Pierini Associated with Borrelia burgdorferi Infection Successfully Treated with Oral Doxycycline. Ann Dermatol. 2011 Aug. 23(3):352-6. [Medline]. [Full Text].

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

  21. 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].

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

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

 
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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.
Single ovoid patch of atrophoderma on the back of a young adult female.
Atrophic hyperpigmented patch with characteristic “cliff-drop” borders.
 
 
 
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