AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

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 IAPP. Canizares et al believed that IAPP differed sufficiently from morphea to classify it as a distinct entity.

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 IAPP is not known. The small number of cases reported may reflect its asymptomatic nature rather than its true incidence.

International

IAPP 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

IAPP is a benign, asymptomatic disease and is not associated with any significant complications or mortality.

Race

IAPP is more common in whites and is rarely reported in blacks or Asians.

Sex

IAPP is more frequently encountered in women than in men. 

Age

IAPP 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.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

History

• IAPP 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. The face, hands, and feet are usually spared. Distribution is often symmetric and bilateral; however, reports have described solely unilateral cases.
• 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

• IAPP 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 IAPP.
• Buechner and Rufi⁵ 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.

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

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 IAPP. Many consider IAPP to be an atrophic stage of morphea.

Since the original description, much debate has occurred regarding whether IAPP 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 IAPP 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. IAPP 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 IAPP, but these conditions lack the pigmentation seen in persons with IAPP.  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 IAPP 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 IAPP 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

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Lab Studies

• Routine baseline studies of the blood and urine may help to exclude other conditions, but they do not help in the diagnosis of IAPP.
• 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.⁶

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. CD34 dermal dendrocytes are reduced, just as in morphea.

TREATMENT

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Medical Care

• No treatment is consistently effective, but some patients respond to topical corticosteroids, antibiotics, or antimalarials.
• Results of medical treatment with antibiotics have been inconclusive. In patients with new early-stage IAPP, especially those with a positive B burgdorferi antibody titer, the standard recommended therapy for Lyme disease is suggested. A retrospective evaluation of 25 patients treated with oral penicillin (2 million IU/d) or oral tetracycline (500 mg 3 times/d) for 2-3 weeks showed clinical improvement with no new active lesions in 20 patients. The same study also showed no progressive disease in 4 of 6 patients who did not receive treatment.
• Anecdotal reports have describe beneficial treatment with the use of hydroxychloroquine⁷ and potassium aminobenzoate.

Surgical Care

• Surgical treatment has generally not been helpful in improving the appearance of the atrophied skin.
• Arpey et al⁸ showed the Q-switched alexandrite laser (755 nm) to be effective in diminishing the hyperpigmentation by 50% after 3 treatments in one case. The improvement was suggested to be caused by reduction of the number, size, and volume of melanosomes.

FOLLOW-UP

Section 7 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Further Outpatient Care

• Once the diagnosis is established, outpatient care consists of following up for the development of new lesions and the involvement of new areas. Ultrasonography may be used for diagnosis and follow-up.

In/Out Patient Meds

• No medications must be ordered. See Medical Care.

Complications

• IAPP is typically a benign, asymptomatic condition; no complications have been reported.

Prognosis

• IAPP has no known effect on the patient's overall health.

Patient Education

• Excessive sun exposure may cause the more deeply pigmented lesional skin to become darker, resulting in further uneven discoloration of the skin. This may be perceived as a serious cosmetic problem.

MISCELLANEOUS

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Medical/Legal Pitfalls

• Thorough history and complete physical examination to look for underlying problems should avoid medicolegal pitfalls.

ACKNOWLEDGMENTS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

We gratefully acknowledge the contributions of Dr. Richard H. Musgnug, author of the previous edition of this article.

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Media file 1:  Early lesion demonstrating diagnostic "cliff-drop" border to atrophy. Courtesy of Joe Eastern, MD.
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Media type:  Photo

Media file 2:  Older lesion showing typical pigmentation and classic "cliff-drop" border. Courtesy of Joe Eastern, MD.
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Media type:  Photo

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

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Article Last Updated: Jul 5, 2007