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Ehlers-Danlos sendromu tip IV semptomlarına eşlik eden yaygın elastozis perforans serpijinoza: bir olgu sunumu

Generalized elastosis perforans serpiginosa coexisting with the symptoms of Ehlers-Danlos syndrome type IV: a case report

Journal Name:

  • Gülhane Tıp Dergisi

Publication Year:

  • 2008

Key Words:

Keywords (Original Language):

Author Name
Abstract (2. Language): 
Elastosis perforans serpiginosa is a rare elastic tissue disorder characterized by keratotic papules, which are usually arranged in serpiginous, annular or arcuate patterns. It is usually observed in young adults. Associations with systemic disorders, such as pseudoxanthoma elasticum, EhlersDanlos syndrome, Marfan syndrome and osteogenesis imperfecta have been reported. Herein a case of generalized reactive elastosis perforans serpiginosa simultaneously diagnosed to have Ehlers-Danlos syndrome type IV with translucent skin, common atrophic scars, postinflammatory hyperpigmentation is presented because of the rare coexistence of these two entities.
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Abstract (Original Language): 
Elastozis perforans serpijinoza, serpijinöz, anüler ya da arklar oluşturmuş, keratotik papüllerle karakterize nadir bir elastik doku hastalığıdır. Sıklıkla genç erişkinlerde gözlenir. Psödoksantoma elastikum, Ehlers-Danlos sendromu, Marfan sendromu ve osteogenezis imperfekta gibi sistemik hastalıklarla birliktelikleri de bildirilmiştir. Burada yaygın bir reaktif elastozis perforans serpijinoza olgusu, translüsent görünümde deri, yaygın atrofik skar ve postinflamatuvar hiperpigmentasyonlar nedeniyle aynı zamanda Ehlers-Danlos sendromu tip IV olarak tanımlanmış ve çok nadir görülen birliktelikleri nedeniyle sunulmaya değer bulunmuştur.
FULL TEXT (PDF): 
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  • 3
213-216
  • Turkish

REFERENCES

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