The Bazex syndrome-follicular atrophoderma, basal cell carcinomas, hypotrichosis, hypohidrosis and some minor skin abnormalities-is described in a Finnish family.Local hypohidrosis was confirmed by measuring the water evaporation rate on the forehead skin. In histological samples from the forehead skin no sweat glands were found. The sweat response due to exercise or metacholine stimulation was slight or negligible.Histologically and ultrastructurally, follicular anomalies were a fundamental feature in the various skin lesions.The Bazex syndrome is a dominantly inherited skin disorder with follicular atrophoderma being the most consistent feature. The patients usually exhibit one or several additional features: multiple basal cell carcinomas, hypotrichosis and localized or general hyphohidrosis. The abnormality was first described by Bazex, Dupre & Christol (1966) in a French family and later by several other authors.We report the syndrome in a Finnish family with five affected members. The case histories, the pedigree and this histological findings are presented. The clinical impression of local hypohidrosis was confirmed by measuring the sweat secretion rates of the patients.
Case reports
Case IA 13 year-old boy had had since birth numerous small, sharp pits on the back of the hands, the upper arms and the knees. At the age of 3 he was treated, as an inpatient, for atopic
50 patients with polymorphous light eruptions were treated with a β-carotene-canthaxanthin combination, Ro 8–8427 (Phenoro®) at a daily dose of 100 mg or placebo for a duration of 10 weeks in a double-blind, randomized, cross-over trial. The effect of treatment was evaluated clinically on the basis of occurrence of pruritus, erythema and eruptions as well as subjectively through the patients own opinion about the condition of their skin. Excellent results after treatment with Ro 8–8427 were achieved in 27 cases and good results in 9, regardless of whether this preparation was the first treatment or the second. The corresponding figures after placebo were 16 and 12, respectively. Similarly, moderate results were achieved in 6 cases after Ro 8–8427 and in 9 cases after placebo. Poor results were achieved in only 3 cases after Ro 8–8427 as compared with 8 after placebo. Only a few, mild side effects were observed during the treatment periods.
A 35-year-old machinist developed hand eczema from contact allergy to dibutylthiourea, a compound found in a paint and glue remover. Light and electron microscopy of his positive patch test was consistent with allergic contact dermatitis, showing Langerhans cell-mononuclear cell contacts in the epidermis.
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