Unilateral Enophthalmos in Linear Scleroderma: A Case Report

선상피부경화증 환자에서 보이는 일측성 안구함몰

  • Cho, So-Hyun (Department of Radiology, Gachon University, Gil Hospital) ;
  • Hwang, Hee-Young (Department of Radiology, Gachon University, Gil Hospital) ;
  • Choi, Hye-Young (Department of Radiology, Gachon University, Gil Hospital) ;
  • Kim, Hyung-Sik (Department of Radiology, Gachon University, Gil Hospital)
  • 조소현 (가천의과학대학교 길병원 영상의학과) ;
  • 황희영 (가천의과학대학교 길병원 영상의학과) ;
  • 최혜영 (가천의과학대학교 길병원 영상의학과) ;
  • 김형식 (가천의과학대학교 길병원 영상의학과)
  • Received : 2010.02.23
  • Accepted : 2010.03.25
  • Published : 2010.08.01

Abstract

Linear scleroderma is an uncommon subtype of localized scleroderma, which is characterized by a linear streak that crosses dermatomes and is associated with the tracking of fibrosis from the skin into deeper tissues, including muscle and fascia. A severe form of this condition sometimes causes growth atrophy of bone and supporting tissue in the affected area. Enophthalmos as a manifestation of linear scleroderma is very rare and occurs due to the replacement of orbital fat and muscle with collagen, which finally leads to atrophy of the affected orbit. This report introduces imaging findings of two cases of enophthalmos caused by linear scleroderma.

선상피부경화증은 국한피부경화증의 아형 중 하나로서, 피부분절을 가로지르는 선상선조와 근육 등의 심부 조직까지 침범하는 섬유화를 특징으로 하는 질환이다. 증상 발현의 하나로써 안구 함몰이 나타날 수 있으나 매우 드물며 안와의 지방과 근육 조직을 콜라겐으로 대체하여 안와의 위축을 일으켜 발생한다. 본 증례 보고는 두 명의 환자를 통하여 선상피부경화증에서 동반된 안구함몰과 영상의학적 소견을 소개하고자 한다.

Keywords

References

  1. Edward H, localized scleroderma. Firestein GS, Budd RC, Harris ED, Mclnnes IB, Ruddy S, Sergent JS. Kelly's textbook of Rhematology. 8th ed. Philadelphia, PA: Saunders Elsevier, 2009;1-1
  2. Goldman L. localized scleroderma. Goldman L, Ausiello DA. Cecil Textbook of Medicine. 23th ed. Philadelphia, PA: Saunders Elsevier, 2008;1-1
  3. Athanasiov PA, Prabhakaran VC, Selva D. Non-traumatic enophthalmos: a review. Acta Ophthalmologica 2008;86:356-364 https://doi.org/10.1111/j.1755-3768.2007.01152.x
  4. Fernando BS, Cannon PS, Tumuluri K, Cook AE. Linear scleroderma as a rare cause of enophthalmos: a case report. J Med Case Reports 2007;1:179 https://doi.org/10.1186/1752-1947-1-179
  5. Kirkali PA, Kansu T, Sanac AS. Unilateral enophthalmos in systemic scleroderma. J Clin Neuroophthalmol 1991;11:43-44
  6. Ramboer K, Demaerel P, Baert AL, Casteels I, Dralands G. Linear scleroderma with orbital involvement: follow up and magnetic resonance imaging. Br J Ophthalmol 1997;81:90-91 https://doi.org/10.1136/bjo.81.1.90