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Congenital atrophoderma of Pasini and Pierini.
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MedLine Citation:
PMID:  16479086     Owner:  NLM     Status:  MEDLINE    
Idiopathic atrophoderma of Pasini and Pierini is a form of dermal atrophy of unknown etiology, usually affecting women during their adolescence and young adulthood. A 2-yr-old girl was presented with erythematous atrophic lesion on the right shoulder, which appeared from birth. The histologic findings were consistent with atrophoderma. This patient, to the best of our knowledge, is the first case of atrophoderma with an onset since birth.
Sung Kwon Kim; Seung Ho Rhee; You Chan Kim; Eun So Lee; Hee Young Kang
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of Korean medical science     Volume:  21     ISSN:  1011-8934     ISO Abbreviation:  J. Korean Med. Sci.     Publication Date:  2006 Feb 
Date Detail:
Created Date:  2006-02-15     Completed Date:  2006-04-10     Revised Date:  2013-06-07    
Medline Journal Info:
Nlm Unique ID:  8703518     Medline TA:  J Korean Med Sci     Country:  Korea (South)    
Other Details:
Languages:  eng     Pagination:  169-71     Citation Subset:  IM    
Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.
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MeSH Terms
Atrophy / congenital,  metabolism
Child, Preschool
Collagen / metabolism
Erythema / pathology
Skin / pathology*
Reg. No./Substance:

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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Journal Information
Journal ID (nlm-ta): J Korean Med Sci
Journal ID (publisher-id): JKMS
ISSN: 1011-8934
ISSN: 1598-6357
Publisher: The Korean Academy of Medical Sciences
Article Information
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Copyright ? 2006 The Korean Academy of Medical Sciences
Received Day: 26 Month: 10 Year: 2004
Accepted Day: 20 Month: 12 Year: 2004
Print publication date: Month: 2 Year: 2006
Electronic publication date: Day: 20 Month: 2 Year: 2006
Volume: 21 Issue: 1
First Page: 169 Last Page: 171
ID: 2733969
DOI: 10.3346/jkms.2006.21.1.169
PubMed Id: 16479086

Congenital Atrophoderma of Pasini and Pierini
Sung Kwon KimA1
Seung Ho RheeA1
You Chan KimA1
Eun-So LeeA1
Hee Young KangA1
Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.
Correspondence: Address for correspondence: Hee Young Kang, M.D. Department of Dermatology, Ajou University School of Medicine, 5 Wonchon-dong, Yeongtong-gu, Suwon 443-721, Korea. Tel: +82.31-219-5190, Fax: +82.31-219-5189,


Idiopathic atrophoderma of Pasini and Pierini (IAPP) is a localized form of dermal atrophy seen typically seen as several oval, hyperpigmented, atrophic plaques developing on the trunk. The lesion usually affects women during their adolescence and young adulthood (1). In this report, we present an unusual case of atrophoderma of Pasini and Pierini with an onset since birth.


A 2-yr-old girl was presented with erythematous atrophic lesion on the right shoulder, which appeared since birth. The size of lesion was slowly growing but the shape, color, or texture of the lesion had not changed since first being discovered. Her family and medical history was unremarkable. On physical examination, she had slightly depressed erythematous plaques on the right shoulder (Fig. 1). There was no induration on the lesion. Punch biopsy for microscopic examination was done from the lesional and adjacent normal-appearing skin for comparison. Histopathological examination of a specimen from the involved skin showed markedly reduced dermal thickness when compared with adjacent normal skin (Fig. 2A, B). The collagen bundles in the dermis were slightly homogenized in appearance (Fig. 2C). The elastic tissue stain showed no abnormality.


IAPP is a form of dermal atrophy, which mostly affects women during their adolescence and young adulthood (2). In one report, of the 40 cases 26 were women and 14 were men, giving a female/male ratio of 2:1. The mean age of patients was 30 yr, although patients have been as young as 7 yr and as old as 66 yr at initial presentation. And 8 of the 40 cases developed before the age of 13 yr (3). However, none have been reported to occur with congenital lesion like our patient. The lesions are single or multiple, usually round or ovoid in shape. The affected skin is usually brown or blue to violet in color and the surface of the skin is normal in appearance with a lack of induration (4). It usually occurs on the trunk, especially on the back and lumbosacral region. Other frequently involved sites are the chest, arms, and abdomen (5). It occurs usually bilaterally and symmetrically in distribution (6). However, unilateral involvement like our case has been reported (7).

Different opinions have been suggested whether IAPP is a variant of morphea or a separate distinct entity. Recently IAPP has been regarded as a superficial form of localized morphea. It is favored by similar clinical and histological features. Lesions of "burn-out" morphea may be atrophic similar to that of IAPP (8) and some histologic similarities such as mild sclerosis and collagen homogenization exist in a few cases (1, 9). There was a case report of a patient with typical IAPP who developed progressive systemic sclerosis (10). However, whether this disorder is an end stage of morphea is unclear. Some clinicians regard IAPP as a distinct entity on the basis of the following reasons. IAPP does not show the typical lilac ring of morphea and IAPP progresses for a longer duration than morphea (1, 8). In addition, the lesions of IAPP are characterized by atrophy without any indurations, whereas morphea begins with indurations and ultimately results in atrophy (1, 11). Moreover, histological findings such as dermal atrophy and usual absence of dermal sclerosis of IAPP are different from those of morphea (1). A report that analyzed glycosaminoglycans in IAPP and morphea showed decreased amount of glycosaminoglycans in IAPP but increased amount in morphea, which suggests that glycosaminoglycans metabolism in IAPP may be unique and quite different from that in morphea (12). From this point of view, our patient could be diagnosed as IAPP. She had atrophic lesions without indurations. And the histological findings showed a significant decrease in dermal thickness when compared with adjacent normal skin, which were consistent with the findings of IAPP.

In summary, IAPP can be presented with a congenital onset and thus should be considered in the differential diagnosis of congenital skin lesions.

1. Canizares O,Sachs PM,Jaimovich L,Torres VM. Idiopathic atrophoderma of Pasini and PieriniAMA Arch DermatolYear: 1958774258
2. Pullara TJ,Lober CW,Fenske NA. Idiopathic atrophoderma of Pasini and PieriniInt J DermatolYear: 1984236436456396245
3. Stanislaw AB,Theo R. Atrophoderma of Pasini and PieriniJ Am Acad DermatolYear: 1994304414468113457
4. Catherine M,Julie P. Jean LB,Joseph LJ,Ronald PRIdiopathic Atrophoderma of Pasini and PieriniDermatologyYear: 2003Vol.2New YorkMosby15441548
5. Beuchner SA,Rufli T. Atrophoderma of Pasini and Pierini: clinical and histopathological findings and antibodies to Borrelia burgdorferi in thirty four patientsJ Am Acad DermatolYear: 1994304414468113457
6. Jaworsky C. Elder D,Elenitsas R,Jaworsky C,Johnson BConnective tissue diseasesLever's Histopathology of the SkinYear: 19978th ednPhiladelphiaLippincott-Raven Co.278279
7. Iriondo M,Bloom RF,Neldner KH. Unilateral atrophoderma of Pasini and PieriniCutisYear: 19873969703802912
8. Poche GW. Progressive idiopathic atrophoderma of Pasini and PieriniCutisYear: 1980255035067379582
9. Miller RF. Idiopathic atrophodermaArch DermatolYear: 1965926536605846320
10. Bisaccia EP,Scarborough DA,Lowney ED. Atrophoderma of Pasini and Pierini and systemic sclerodermaArch DermatolYear: 1982118127059194
11. Eshelman O. Idiopathic atrophoderma of Pasini and PieriniArch DermatolYear: 196592737738
12. Yokoyama Y,Akimoto S,Ishikawa O. Disaccharide analysis of skin glycosaminoglycan in atrophoderma of Pasini and PieriniClin Exp DermatolYear: 20002543644011012603

Article Categories:
  • Case Report

Keywords: Atrophy, Dermis, congenital.

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