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
The local sweating response to thermal stress (mean ambient temperature 33 degrees C) was assessed under resting conditions on the non-eczematous back skin of 26 young men with atopic dermatitis (AD) and in 22 non-atopic controls with other dermatoses. The baseline (transepidermal) water loss was separately determined at room temperature (mean 23.6 degrees C) to calculate the pure sweat loss. A gravimetric collecting method was used for the measurements at 40, 60 and 80 min. In the heated room the sweat loss in AD patients was significantly lower at all time intervals. The cumulative sweat loss was 50-60% lower in AD patients than in the controls (P less than 0.02). Subjects with dry AD skin had a lower sweat loss than subjects with normal-looking skin. Compared with controls the sweat loss in AD patients was lowest at 40 min, suggesting a retarded sweating response. Half of the patients with AD and half of the controls had active participation in sports, and showed a greater sweat loss compared to the non-sporting subjects in the same group.
We challenged in a double‐blind manner 46 pollen‐allergic patients with 30 g of honey and another 32 patients with a placebo (30 g of syrup). Minor, mostly subjective, symptoms were seen in or reported by 26% of those challenged with honey and 41% of those challenged with placebo. In no case could the symptoms with certainty be related to the challenge. Eight commercially available honeys were examined for allergen activity by RAST inhibition and immunospot methods. Both pollen and insect allergen activity was found in all honeys, and they could cause allergic reactions. However, no serious or even obvious reaction occurred in pollen‐allergic patients challenged with honey.
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