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Atrophoderma of Pasini and Pierini Workup

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

Approach Considerations

The laboratory, imaging and histological findings of idiopathic atrophoderma of Pasini and Pierini are non-specific and the diagnosis is typically made on the basis of clinical features. 

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Laboratory Studies

Routine baseline studies of the blood and urine may help to exclude other conditions, but they do not help in the diagnosis of idiopathic atrophoderma of Pasini and Pierini.[14]

Screening tests such as the enzyme-linked immunosorbent assay may be performed to detect anti– B burgdorferi antibodies.

One anecdotal case report from 2012 advocates thyroid gland evaluation in all patients with sclerodermalike disorders because of pathophysiological links between cutaneous fibrosis and the thyroid gland.[15]

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Imaging Studies

The thickness of the dermis and subcutis may be measured using magnetic resonance imaging or 13-MHz B-mode ultrasonography.[16]

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Procedures

Skin biopsy is not always necessary to make the diagnosis; however, it may be useful to exclude other entities.

Dermal atrophy is more easily evaluated with wedge excision than punch biopsy. An elliptical biopsy specimen is sectioned longitudinally from an area that includes normal skin and the cliff-drop border of the lesion. If dermal atrophy is present, the transition between normal dermis to atrophied dermis is discernible.

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Histologic Findings

Histopathologic changes, often minimal and nondiagnostic, consist of a decrease in the size of the dermal papillae with flattening of the rete pegs. The epidermis is usually normal or slightly atrophic. Melanin may be increased in the basal layer. Interstitial edema and a mild perivascular infiltrate consisting of lymphocytes and histiocytes may be present. Collagen bundles show varying degrees of homogenization and clumping in the mid and reticular dermis. When compared with adjacent normal skin, dermal thickness is reduced. The sweat glands, pilosebaceous units, and appendages are not affected.

In most series, no abnormalities in elastic fibers have been observed with either elastic tissue staining[12] or electron microscopy.[17] However, a 2008 study described a spectrum of histopathological findings ranging from normal to severe diminution and fragmentation of elastic fibers, with 35.3% of cases showing moderate-to-severe reduction and fragmentation.[10]

If preexisting patches show sclerodermatous changes, histology may reveal varying degrees of collagen sclerosis resembling morphea. Direct immunofluorescence of early lesions may show nonspecific immunoglobulin M and C3 staining in the dermal papillary blood vessels or at the dermoepidermal junction.[18] CD34 dermal dendrocytes are reduced, just as in morphea.

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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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