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Pediatric Radiology

, Volume 18, Issue 1, pp 62–66 | Cite as

Radiological features in congenital erythropoietic porphyria (Gunther's disease)

Report of 3 cases
  • M. Levesque
  • P. Legmann
  • A. Le Cloirec
  • J. C. Deybach
  • Y. Nordmann
Originals

Abstract

Gunther's disease or congenital erythropoïetic porphyria is a rare and severe disorder comprising cutaneous and haemolytic symptoms. Photocutaneous lesions are responsible for scleroderma-like calcifications and deformities of the extremities visible on X-rays. Hemolytic manifestations lead to diffuse major osteopenia. Soft tissue calcifications of the fingers can be seen even in young patients. One case reported here is the first illustration of intracranial calcifications located on dura-mater and calvarium.

Keywords

Public Health Soft Tissue Young Patient Tissue Calcification Radiological Feature 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • M. Levesque
    • 1
    • 3
  • P. Legmann
    • 1
  • A. Le Cloirec
    • 1
  • J. C. Deybach
    • 2
  • Y. Nordmann
    • 2
  1. 1.Department of RadiologyUniversity Xavier BichatColombesFrance
  2. 2.Department of Biochemistry of Hôpital Louis MourierUniversity Xavier BichatColombesFrance
  3. 3.Department of RadiologyHôpital Louis MourierColombesFrance

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