Darier’s disease

Darier's-disease

Don’t forget Darier’s disease in spite of its rarity !
1. Autosomal dominant inheritance.
2. Usual onset before 30 years.
3. Dirty warty papules mainly in seborrheic areas. The skin may have coarse sandpaper texture.
4. Photoexacerbation of the rash is common.
5. Palmoplanter pits.
6. Characteristic V-shaped nicks at the free edge of the nails.
7. Keratosis follicularis (Acrokeratosis verruciformis) on hands and feet.
8. Red mucous membranes and occasinally have cobblestone appearance.
9. A characteristic “focal acantholytic dyskeratosis” picture on histopathology.
10. Retinoids (Acitretin/ Isotretinoin) are usually effective in severe cases.