Keratosis follicularis is a disorder characterized by erythematous scaling papules most pronounced in sun exposed areas. Darier's disease is slowly progressive.
Papules erupt and coalesce into cobblestone plaques.
When the flexures are involved, there may be fissuring evident. When fissures are present, there is often a significant bacterial overgrowth.
The papules can be found on the palms and soles, as well as the mucosal surface. The characteristic nail change consists of red and white longitudinal bands, with a nick at the distal edge of the nail, at the end of these bands.
Darier's disease shows a similar histology to, and can coexist with, chronic benign familial pemphigus (Hailey-Hailey Disease).
- In mild disease attempts should be made to minimize irritation of the skin. Gentle cleanses and keeping the skin gently moisturized; avoidance of over-heating
- Tazarotene gel as well as topical isotretinoin and Tretinoin have been helpful. The irritation caused by these products may be inhibited by the combination of topical corticosteroids
- Etretinate and Acitretin have been shown to be useful in more extensive cases
- Topical 5FU has been shown to be of value, especially if it is combined with systemic retinoids.
- Cyclosporine has been utilized with benefit in those who have accompanying widespread eczematous eruption.
- Oral contraceptive pills may reduce the pre-menstrual flare of this condition.
- Essential fatty acids have been documented to be helpful in case reports.
- Laser destruction of localizes persistant areas using CO2 and erbium:YAG laser have also been found to be useful.
- Excision of persistent hypertrophic plaques is also to be considered.
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