- A dictionary of medical eponyms

Darier-White syndrome

Alternative eponyms

  • Darier's disease
  • Darier's syndrome
  • Darier-White disease
  • Lutz-Darier syndrome
  • White's disease
  • White’s syndrome

Related people

A skin disease with onset in childhood.

Description

A skin disease with onset in childhood, usually around the age of ten years, rarely in infants. Very long list of symptoms include many confluent flesh-coloured keratotic papules forming greasy and vegetating crusted areas, covering the seborrheic areas of the body: the chest, ears, nasolabial folds, scalp, neck, back, abdomen, and groin. On removing the lesion, a porelike opening is revealed. Eventually, the lesions coalesce into large, sometimes thick plaques to form hypertrophic, foul-smelling masses. Both sexes affected. Aetiology unknown. The condition tends to be aggravated by sunlight. Autosomal dominant inheritance.

Some authors consider this as a variant of Hopf’s syndrome (acrokeratosis verruciformis). See under Gustav Hopf, German dermatologist, 1900-1979.

Bibliography

  • H. C. Lutz:
    De l’hypertrophie générale du système sebacé.
    Paris, Thèse No. 65, 1860.
  • J. C. White:
    A case of keratosis (ichtyosis) follicularis.
    Journal of Cutaneous and Genitourinary Diseases, Chicago, 1889, 7: 201-209.
  • F. J. Darier:
    De la psorospermose folliculaire végétante. Étude anatomo-pathologique d'une affection cutanée non décrite ou comprise dans le groupe des acnés sebacées, cornées, hypertrophiantes, des kératoses (ichtyoses) folliculaires, etc.
    Annales de dermatologie et de syphilographie, Paris, 1889, 10: 597-612.
  • F. J. Darier et Thibault:
    De la psorospermose folliculaire végétante- Étude anatomo-pathologique d’une affection cutanée non décrite ou comprise dans le groupe des acnés sébacées, cornées hypertrophiantes, des kératoses (ichtyoses) folliculaires.
    Thèse de Paris. 1889.

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