Treatment of Dyskeratosis Follicularis Darier with the Retinoic Acid Derivative Ro 10–9359 (Tigason®)

Hereditary disorders of keratinization may be a considerable handicap. Oral treatment with retinoids has been shown to be effective in many of these diseases. In the group of ichthyoses, the best results can be obtained in the various types of nonbullous congenital ichthyosis (erythrodermic autosomal recessive lamellar ichthyosis, nonerythrodermic autosomal recessive lamellar ichthyosis, autosomal dominant lamellar ichthyosis). It should be borne in mind, however, that retinoid therapy alone cannot lead to a complete response of these forms of ichthyosis and that this treatment cannot replace an appropriate topical treatment. During continuous treatment with etretinate a reduction of the dosis to 0.5 mg/kg is often necessary. Etretinate treatment of bullous congenital ichthyosiform erythroderma is more difficult, and it is advisable to begin with a low dosis of 0.25–0.5 mg/kg. The epidermolytic form of palmoplantar keratoderma is in our opinion no indication for retinoid treatment which seems to result inevitably in large erosions. Good or excellent results have been seen in other forms of palmoplantar keratoderma including mal de Meleda, Papillon-Lefèvre syndrome, erythrokeratodermia variabilis, verrucous epidermal nevi, Darier disease and pityriasis rubra pilaris. In patients with Darier disease it is wise to begin with a relatively low dosage of 0.5 mg/kg and to adjust the dosage to the further course of the disease. The same is true for the ichthyosis seen in the Netherton syndrome, which may be either a diffuse hyperkeratosis or ichthyosis linearis circumflexa. In view of the fact that any inherited keratinization disorder requires long-term treatment, the risk of bone toxicity should be carefully weighed against the benefit of this therapy. The results so far obtained indicate that the effect of etretin is comparable to that of etretinate in the treatment of inherited keratinization disorders. Intermittent therapy should be tried whenever possible. A combination therapy seems reasonable in pityriasis rubra pilaris of the adult type. We have seen good results by combination with PUVA treatment. Autosomal dominant ichthyosis vulgaris and X-linked recessive ichthyosis are inappropriate to treat with oral retinoid therapy because these diseases are too mild. Papillomatous epidermal nevi should also be excluded because they do not respond to the drug. Hailey-Hailey disease may even be worsened by this treatment. According to our experience, oral retinoid therapy has no effect in monilethrix.

Section: Darier Diseasesupporting

confidence: 82%

Retinoids in Disorders of Keratinization: Their Use in Adults

Happle

Kerkhof²,

Traupe³

1987

“…It supports previous reports on the clinical ef ficacy of etretinate in Darier's disease, pustu losis palmo-plantaris, psoriasis, and eczema kcratoticum [1][2][3][4][5], although our percentage of patients experiencing a 'good effect' is not as high as found in prospective studies of etretinate. Our retrospective study does not allow us clearly to measure the influence of the topical therapy on the results.…”

Section: Discussionsupporting

confidence: 91%

Patients’ Acceptance of Etretinate Therapy

Christiansen

Holm

Reymann³

et al. 1984

Self Cite

From January 1976 to April 1982 etretinate was used to treat patients with morbus Darier (25 patients), pustulosis palmo-plantaris (72 patients), psoriasis (79 patients), and hyperkeratotic eczema of hands and feet (eczema keratoticum; 41 patients). Dosage of etretinate ranged between 0.5 and 1.0 mg/kg/day. In a retrospective survey at April 1 1982, we observed a beneficial effect of the drug in all patients with morbus Darier, in 85% of patients with pustulosis palmo-plantaris, in 69% with psoriasis, and in 93% with eczema keratoticum. Many patients experienced side effects. Within 9 months more than half of the patients had stopped treatment primarily due to side effects, except patients with morbus Darier, whose median duration of treatment was 34 months with a range from 7 to 60 months. We will recommend that etretinate is used during short-term or intermittent courses of therapy, except in patients with morbus Darier, where a low dosage of etretinate is able to give a beneficial clinical effect.

“…The results obtained of Tigason treatment of psoriasis and Darier's disease in this Department have already been described [1,2] and the present paper gives a brief report on our experience after 2 years of ther apeutic experiments in conditions such as pustulosis palmo-plantaris (PPP) and eczema keratoticum manuum (EKM).…”

Section: Introductionmentioning

confidence: 82%

“…Prior to and during treatment with Tigason the patients were carefully checked with regard to possible disturbances in bone marrow, liver and kidney function according to the same principles as those described in previous reports [1,2], Dosage varied from 25 to 50 mg/24 h and only in a few cases was the dose raised to 75 mg/24 h. Topical treatment was limited to neutral preparations such as moisture cream or cold cream. However, in patients who were treated for 6 months or more, Tigason was combined with steroid cream, salicylic vaseline or dithranol preparations, depending on the severity of each individual case.…”

Section: Methodsmentioning

confidence: 99%

Two Years’ Experience with Tigason Treatment of Pustulosis palmo-plantaris and Eczema keratoticum manuum

Reymann¹

1982

Self Cite

The results are presented of 2 years’ experiments with Tigason® treatment of pustulosis palmo-plantaris (19 patients) and eczema keratoticum manuum (15 patients). These two skin diseases are characterized by being extremely resistant to conventional dermatological therapy, but responding immediately to treatment with Tigason. The rather high frequency of side-effects of the drug (dryness of mucosae, loss of hair, etc.) complicates the treatment but in about 50% of patients continuous or intermittent treatment over several years proved to be really beneficial.