Darier's disease and other dyskeratoses: Responce to retinoids

Retinoid therapy represents the treatment of choice for severe inherited disorders of keratinization. This paper reviews our experience of acitretin, compares acitretin with etretinate and defines guidelines for treatment. Forty-six children have received acitretin since 1992 in our hospital: 29 children had either lamellar ichthyosis (nine), non-bullous ichthyosiform erythroderma (five), bullous ichthyosiform erythroderma (four), Sjögren-Larsson syndrome (three) or another rare condition (eight). The other 17 children who had psoriasis (16) and extensive viral warts (one), were excluded. Data on efficacy and tolerability of retinoid therapy were available for all but one patient. The cumulative follow-up was 472 months for acitretin. The mean (+/- standard deviation) optimal dosage for acitretin was 0.47 +/- 0.17 mg/kg per day, and this did not significantly differ between disorders. The overall improvement was considerable, with only three patients responding poorly. Mild to moderate mucocutaneous dryness was frequent. Minor abnormalities of liver function tests (four patients) and triglycerides (one patient) never led to changes of therapy. Irreversible side-effects did not occur. Acitretin therapy for children with inherited keratinization disorders is best started at 0.5 mg/kg per day. It represents a safe and effective treatment, provided that the minimal effective dose is maintained and that side-effects are carefully monitored. When switching from etretinate to acitretin, a 20% reduction is recommended if the etretinate dose is over 0.75 mg/kg per day or if side-effects are dose limiting. Otherwise the same dose can be used.

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“…21. The results are listed as the number of patients who reporteid mild, moderate or severe side-effects at any one time.…”

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confidence: 99%

An appraisal of acitretin therapy in children with inherited disorders of keratinization

Lacour

MEHTA-NIKHAR

Atherton

et al. 1996

British Journal of Dermatology

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“…Treatment remains unsatisfactory although aromatic retinoids, (isotretinoin and etretinate) have benefited some patients. 11 We have identified a large family segregating Darier's disease and have begun a scan of all chromosomes with DNA markers in an effort to locate the causative gene. This report summarizes our strategy and the loci tested thus far.…”

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confidence: 99%