eMedicine Specialties > Dermatology > Diseases of the Dermis

Atrophoderma of Pasini and Pierini: Differential Diagnoses & Workup

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

Differential Diagnoses

Anetoderma
Lichen Sclerosus et Atrophicus
Morphea
Postinflammatory Hyperpigmentation

Other Problems to Be Considered

Late-stage morphea, a burned-out stage of localized scleroderma, systematized epidermal nevus, postinflammatory hyperpigmentation secondary to herpes zoster, and atrophoderma of Moulin may resemble idiopathic atrophoderma of Pasini and Pierini. Many consider idiopathic atrophoderma of Pasini and Pierini to be an atrophic stage of morphea.

Since the original description, much debate has occurred regarding whether idiopathic atrophoderma of Pasini and Pierini is a distinct entity or an atrophic, nonindurated variant of morphea, despite differences in the origin, development, and outcome of the lesions. The existence of a relationship between idiopathic atrophoderma of Pasini and Pierini and morphea is supported by the striking clinical and histological similarities seen at sites of regressing plaques of morphea. Morphea characteristically begins as a discrete circumscribed, erythematous-to-sclerotic plaque, often with a white center and characteristic peripheral lilac rim. Idiopathic atrophoderma of Pasini and Pierini lacks sclerosis, and lesions commonly coalesce over time, producing a moth-eaten appearance that is not consistent with morphea.  
 
From a distance, the skin depression in lichen sclerosis et atrophicus, anetoderma, or resolving panniculitis may resemble a stage of idiopathic atrophoderma of Pasini and Pierini, but these conditions lack the pigmentation seen in persons with idiopathic atrophoderma of Pasini and Pierini.  Anetoderma, also a dermal atrophic process, is easily differentiated by palpation and, with histology studies, loss of dermal elastic fibers.
 
Typical lesions of morphea and idiopathic atrophoderma of Pasini and Pierini may appear in different or adjacent areas on the same individual. These conditions may occur simultaneously or one may precede the other. This observation may support the hypothesis that idiopathic atrophoderma of Pasini and Pierini and morphea are variations in response to the same abnormality.
 
The diagnosis of atrophoderma of Moulin requires that the acquired hyperpigmented linear atrophoderma follows Blaschko lines.

Workup

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

Imaging Studies

  • The thickness of the dermis and subcutis may be measured using magnetic resonance imaging or 13-MHz B-mode ultrasonography.9

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.

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 is 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, with a normal papillary dermis. When compared with adjacent normal skin, dermal thickness is reduced. The sweat glands and the pilosebaceous units are not affected. The appendages are preserved. Elastic fibers appear normal after elastic tissue staining and with electron microscopic studies.
 
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.10 CD34 dermal dendrocytes are reduced, just as in morphea.

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

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  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].

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  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].

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  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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