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Darier’s Disease (Keratosis Follicularis)

What is Darier’s disease (keratosis follicularis) ?

  • Also called Darier-White Disease.
  • Is one of the most common genodermatosis and affects one person out of 30000. It is inherited in an autosomal dominant pattern.

 

 

What is it caused by ?

  • The mutated gene codes for a calcium channel regulator.

 

 

How does it look like on the skin ?

  • On the skin, there are brownish follicular bumps which look greasy and “dirty looking.”
Lésions typiques de la maladie de Darier
Typical lesions of Darier’s disease
  • The bumps appear around puberty in seborrheic (seborrhoeic) areas: midchest, face, behind the ears.
  • The palms show depressions (called pits).
  • Nails have longitudinal white or red nail stripes.
Stries longitudinales des ongles (maladie de Darier)
Longitudinal stripes on the nails (Darier’s disease)

 

 

Is the skin more fragile ?

  • Yes. Widespread infections are more common.

 

 

What is the treatment ?

  • Local treatment with retinoids and keratolytics is quite disappointing.
  • Antibiotics and Antivirals may be used in case of infections.
  • Oral Acitretin seems to show the best results.

 

 

Source of information: here