Dyskeratosis follicularis disease: case reports and review of the literature.

BACKGROUND

Dyskeratosis follicularis (Darier's disease) is rare autosomal dominant disease characterized by the loss of adhesion between epidermal cells and by abnormal keratinization.

METHODS

We performed a retrospective study of all the patients diagnosed with Darier's disease at the Department of Dermatology of Charles Nicolle Hospital of Tunis, between 1971 and 2002.

RESULTS

During the observation period, we identified 12 patients with Darier's disease; five males and seven females with a mean age of 17.36 years. No family history was found in eight patients. Skin lesions in the form of keratotic papules were noted in seborrhoeic areas, essentially the face (nine patients), chest and scalp. Seven patients had nail lesions. UV light exposure exacerbated the disease symptoms in seven cases. The patients were treated with topical and systemic retinoids (six cases).

CONCLUSION

Although Darier's disease has a chronic course, most patients manage to lead a relatively normal life. Treatment is usually unsatisfactory despite much progress in understanding of the underlying abnormalities in Darier's disease.