Darier’s Disease (Keratosis Follicularis)

Dr Christophe Hsu – dermatologist. Geneva, Switzerland

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.”

• 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.

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.

Contributors

Dr Christophe Hsu – dermatologist. Geneva, Switzerland

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