Darier-White syndrome
Related people
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.