Ichthyosis has been classified on a genetic basis. Four varieties--ichthyosiform erythroderma, lamellar ichthyosis, Refsum's syndrome and the Sjögren-Larsson syndrome--are inherited as autosomal recessive traits. One type is inherited as a sex-linked recessive trait while another variety of ichthyosis (with or without atopy), bullous ichthyosiform erythroderma, and very rarely ichthyosis hystrix are due to autosomal dominant genes.

Humans↗

PubMedDate not supplied

Treatment of chronic mucocutaneous candidosis with ketoconazole: a study of 12 cases.

Twelve patients with chronic mucocutaneous candidosis were treated orally with ketoconazole (doses, 200-400 mg daily) for a mean period of six months. Seven of the patients had one of the following abnormalities: congenital endocrinopathy syndrome, an autosomal recessive or autosomal dominant defect in which candidosis is not associated with endocrinopathy, or the malabsorption syndrome. All patients had fungal infections of the mouth, and 11 had onychomycosis. Two patients were also infected with dermatophytes. At the end of treatment, 10 patients were cured of oral infection, and 11 with nail infections showed significant improvement. Marked improvement of hand and foot infections was also recorded. Patients infected with dermatophyte fungi had the poorest responses to therapy. The mean (+/- SD) MIC for isolates of Candida albicans from eight patients was 0.95 (+/- 0.78) microgram/ml. Clinical and biochemical monitoring showed no toxicity, and no resistant fungi emerged during treatment. Results of this initial study of ketoconazole for treatment of severe and recalcitrant superficial infections indicate the need for further assessment of this drug, which appears to offer a simple, nontoxic, and effective treatment of fungal infections.

Adolescent↗