Oral retinoid (Tigason R etretinate) therapy in congenital disorders of keratinisation.
We report our experience in Singapore with the use of the new oral Retinoid-etretinate (Tigason RO 10-9359) in 23 patients suffering from various congenital disorders of keratinisation. In this study the drug was administered to 8 patients with Darier's disease, 7 patients with congenital ichthyosis, 4 patients with palmar plantar keratoderma, 2 patients with systematised epidermal naevus and 2 patients with progressive symmetrical erythrokeratoderma. The best results were obtained in patients with ichthyosis where complete clearance was possible. All patients with Darier's except one showed significant improvement. Palmar plantar keratoderma except for one patient gave only fair to minimal improvement. Etretinate was useful in systematised epidermal naevus and progressive symmetrical erythrokeratoderma. Pruritus and cheilitis were the commonest side effect. In two patients (both with Darier's disease) treatment was stopped because of side effects. The side effects were dose related. The histology showed a reduction of the keratin layer but remnants of the original features of the pathology were still present.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D007644:Darier Disease / Q000151:congenital / Q000188:drug therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007057:Ichthyosis / Q000151:congenital / Q000188:drug therapy; D007645:Keratoderma, Palmoplantar / Q000151:congenital / Q000188:drug therapy; D007642:Keratosis / Q000151:congenital / Q000188:drug therapy*; D008297:Male; D009508:Nevus, Pigmented / Q000151:congenital / Q000188:drug therapy