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[Universal dyschromatosis: a familial case]

P Schoenlaub1, J P Leroy, D Dupré

  • 1Service de Dermatologie, Hôpital Laennec, Quimper.

Annales De Dermatologie Et De Venereologie
|December 4, 1998
PubMed
Summary

Universal dyschromatosis is a rare genetic skin disorder with variable presentation. This family study shows it has autosomal dominant inheritance with incomplete penetrance, suggesting localized forms are milder expressions.

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Area of Science:

  • Genetics
  • Dermatology
  • Cell Biology

Background:

  • Universal dyschromatosis is a rare genodermatosis characterized by generalized leucomelanodermia.
  • First recognized in Japan in 1933, its mode of transmission and ultrastructural aspects have been investigated.

Observation:

  • A family presented with universal dyschromatosis, including a 9-year-old girl with insulin-dependent diabetes mellitus and her father.
  • Clinical examination revealed generalized leukomelanoderma with alternating achromatic and pigmented maculae, predominantly on the trunk.
  • Skin biopsy and ultrastructural analysis showed intact pigment transfer and normal melanocyte numbers, suggesting a functional melanogenesis anomaly.

Findings:

  • The family pedigree indicated autosomal dominant inheritance with incomplete penetrance for hereditary leukomelanoderma.

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  • Familial cases illustrated variable clinical presentations, with localized forms appearing as incomplete expressions of the dermatosis.
  • Ultrastructural investigations revealed varying melanocyte activity levels without abnormal pigment production or transfer.

    • Implications:

    • Universal dyschromatosis is a rare genodermatosis with variable expression, potentially encompassing previously described localized forms.
    • Understanding the genetic transmission and variable presentation aids in diagnosing and managing this pigmentary abnormality.
    • Distinguishing universal dyschromatosis from conditions like xeroderma pigmentosum is crucial, particularly noting the absence of photosensitivity.