European Journal of Pediatrics

, Volume 150, Issue 5, pp 343–346 | Cite as

Calcinosis cutis, osteoma cutis, poikiloderma and skeletal abnormalities (COPS syndrome) — a new entity?

  • A. P. Oranje
  • S. M. P. F. de Muinck Keizer-Schrama
  • V. D. Vuzevski
  • M. Meradji
Medical Genetics

Abstract

A 4-year-old boy with subcutaneous tumours is described. These tumours were calcified and had secondary osteoma formation. In addition the patient showed poikiloderma on the face and less prominently on arms and legs. X-ray films of the distal metaphyses of the radius, ulna and tibia revealed irregular mineralisation. Repeated laboratory tests revealed no abnormalities of fat, bone and mineral metabolism. This patient showed a unique combination of symptoms. We propose to call this syndrome: COPS-syndrome (Calcinosis cutis,Os-teoma cutis.Poikiloderma andSkeletal abnormalities).

Key words

Calcinosis Osteoma Poikiloderma Syndrome 

Abbreviation

AHO

Albright hereditary osteodystrophy

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Copyright information

© Springer-Verlag 1991

Authors and Affiliations

  • A. P. Oranje
    • 1
  • S. M. P. F. de Muinck Keizer-Schrama
    • 2
  • V. D. Vuzevski
    • 3
  • M. Meradji
    • 4
  1. 1.Department of Dermatovenereology (Division of Paediatric DermatologyErasmus University Rotterdam and University Hospital Rotterdam-Dijkzigt Sophia, Sophia Children's HospitalRotterdamThe Netherlands
  2. 2.Department of PaediatricsErasmus University Rotterdam and University Hospital Rotterdam-Dijkzigt Sophia, Sophia Children's HospitalRotterdamThe Netherlands
  3. 3.Department of PathologyErasmus University Rotterdam and University Hospital Rotterdam-Dijkzigt Sophia, Sophia Children's HospitalRotterdamThe Netherlands
  4. 4.Department of RadiologyErasmus University Rotterdam and University Hospital Rotterdam-Dijkzigt Sophia, Sophia Children's HospitalRotterdamThe Netherlands

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