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Pseudoxanthoma elasticum
  1. S Laube,
  2. C Moss
  1. Department of Dermatology, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK
  1. Correspondence to:
    Dr S Laube
    University Hospital of North Staffordshire, Department of Dermatology, Hartshill Road, Stoke-on-Trent ST4 7PA, UK;


Pseudoxanthoma elasticum (PXE) is a rare multisystem disorder characterised by progressive calcification and fragmentation of elastic fibres. Recent genetic advances have identified the underlying defect to the ABCC6 gene on chromosome 16p13.1. Patients typically develop cutaneous, ocular, and cardiovascular manifestations but there is considerable phenotypic variability. The skin changes are usually apparent in adulthood, and rarely observed in childhood. Since the prognosis of PXE largely depends on the extent of extracutaneous organ involvement early recognition, intervention and lifestyle adjustments are important to reduce morbidity. First-degree family members should be carefully examined for any cutaneous or ophthalmologic features of PXE.

  • angioid streaks
  • connective tissue disorder
  • disorder of elastic fibres
  • premature atherosclerosis
  • pseudoxanthoma elasticum

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  • Competing interests: none

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