Darier's disease

Darier's disease is a rare disorder of keratinisation with an autosomal dominant pattern of inheritance whose severity fluctuates over time. The first skin lesions typically occur in childhood or adolescence like hyperkeratotic, follicular papules skin-colored or yellow-brown with a predilection for seborrheic areas; ichtyosis, nail changes, palmar pitting, punctate keratoses, hyperkeratotic papules, mucosal lesions may also be seen. We report the case of a 70-year-old man who was admitted in our hospital for extensive hyperkeratotic brown plaques on the chest and back and for multiple disseminated hiperkeratotic brown papules on the margin of the scalp, forehead, ears and the dorsum of the hands. The lesions occurred when he was a teenager. Based on clinical and paraclinical findings, a diagnosis of Darier's disease was established. We tried systemic retinoids but they have not been tolerated. We have used emollients, topical retinoids and corticosteroids with moderate improvement.