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[Diffuse disease of elastic tissue and hyperphosphoremia].

C Francès, D Emilie, B Wechsler

    Annales De Dermatologie Et De Venereologie
    |January 1, 1987
    PubMed
    Summary

    This study details a rare case of diffuse elastic tissue disease linked to hyperphosphatemia, distinct from tumoral calcinosis. The findings reveal similarities to Pseudoxanthoma elasticum (PXE) in elastic tissue abnormalities and arterial calcification.

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    Area of Science:

    • Vascular Biology
    • Metabolic Disorders
    • Dermatopathology

    Background:

    • Pseudoxanthoma elasticum (PXE) is linked to abnormal phosphate-calcium metabolism.
    • Tumoral calcinosis presents with hyperphosphatemia and can exhibit elastopathy resembling PXE.
    • This report describes a novel case of diffuse elastic tissue disease with hyperphosphatemia, without tumoral calcinosis.

    Observation:

    • A 29-year-old Gabonese patient presented with severe arteritis, arterial calcifications, and retinal angioid streaks.
    • Histological examination revealed fragmented and curled elastic fibers in the skin, similar to PXE.
    • Morphometric analysis showed wider, longer elastic fibers and reduced oxytalan/elaunin fibers compared to normal tissue.

    Findings:

    • The patient exhibited hyperphosphatemia due to increased renal tubular phosphate reabsorption.

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  • Arterial histology mirrored that of PXE patients.
  • Elastic tissue abnormalities in the patient were comparable to those in PXE.

    • Implications:

    • This case expands the understanding of elastic tissue disorders and their association with metabolic abnormalities.
    • It highlights a potential link between hyperphosphatemia and PXE-like elastopathy.
    • Further research may elucidate the mechanisms connecting phosphate metabolism and elastic tissue degeneration.