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Digital papillary adenocarcinoma is a rare but well characterized carcinoma of the sweat glands, which apart from very few exceptions is localized in acral skin. This type of sweat gland carcinoma tends to recur locally and may cause delayed metastases in a few cases. We describe the clinical findings and the broad histopathologic spectrum of four cases of this rare adnexal carcinoma and give a short summary of the literature.

Fabry disease is an inherited X-linked lysosomal storage disease due to a genetic defect of the GLA gene which encodes the protein of the enzyme alpha-galaktosidase A. Under normal concentrations this lysosomal enzyme is involved in degradation and catabolism processes of membrane glycosphingolipids in almost all cells of the human organism. The enzyme deficiency leads to a progressive accumulation of globotriaosylceramide (Gb3) in various tissues and organ systems and is responsible for the large variability of the clinical signs and symptoms of the disease. First signs and symptoms such as painful neuropathy (acroparethesia), hypo- or unhidrosis and gastrointestinal disturbances can be found already in childhood and mainly affect quality of life. In the following decades of life, renal cardiac, and cerebrovascular complications occur in hemizygous males and with some delay in a part of heterozygous females as well. If not treated, these complications result in increased morbidity and premature mortality. With the introduction of the enzyme replacement therapy (ERT) in 2001 a causal treatment is available. Published data from clinical trials and observational studies demonstrated that ERT mitigates signs and symptoms of the disease as well as quality of life, and has the potential to reduce mortality. The efficacy of ERT seems to be less pronounced in severe cases of the disease, which makes an early diagnosis and treatment more important in order to prevent or improve the progression of the disease.

Surveys in USA and UK have shown inconsistencies between practice of sweat testing and guidelines of the National Committee for Clinical Laboratory Standards (NCCLS), which could increase the risk of false-positive and -negative results. To determine the current practice of sweat testing in German cystic fibrosis (CF)-centres a survey was performed.A questionnaire was mailed to all 123 German CF-centres that join the German CF-register. The results were compared with the NCCLS-guidelines.88 out of 113 responding centres perform 30-1100 (median 200) sweat tests by pilocarpine iontophoresis per year. The recommended sweat sample time of 20-30 min was accepted by 33% of all centres. Minimum acceptable sweat rates were quoted between 100 and 7500 (median 1000) mg/m (2)/min differing from the recommended 1000 mg/m (2)/min. Osmolality was measured in 3%, sodium in 24% and conductivity in 53% of the centres. Chloride, the only accepted diagnostic measurement by the NCCLS, was investigated by only 58% of all centres. The recommended chloride CF-reference value of60 mmol/l was used in 78% of centres that perform chloride testing.Major areas of inconsistencies between sweat testing practice in German CF-centres and NCCLS-guidelines are sweat collection, analyzing method and reference values. Chloride, the only accepted diagnostic measurement by the NCCLS, was investigated only by 58% of all centres.

Noninvasive investigation of skin functions is increasingly employed in dermatology and cosmetology. It enables one to study aspects of skin functions that cannot always be appreciated by sensory perception. Noninvasive methods permit objective and reproducible investigation of distinct biophysical parameters. In the age of evidence-based medicine this becomes more and more important. Biophysical data can be quantified, analyzed, and stored electronically. In an overview, selected noninvasive techniques of skin function testing are introduced and their relevance in dermatology, dermatopharmacology, and cosmetology is discussed.

We report the history of an infant who presented with hypotonic dehydration and metabolic alkalosis, in whom the diagnosis of cystic fibrosis was made on the basis of investigations for rare cystic fibrosis mutations. Since no other signs and symptoms of the CF disease were present, the finding of the rare mutation D110H on exon 4 of the CFTR-gene was paramount in the delineation of his underlying illness. He is now thriving well with a daily oral substitution of 1-2 grams of sodium chloride. A mild variant of cystic fibrosis has to be considered in infants presenting with unexplained hypoelectrolytemia and metabolic alkalosis. Like in our child, typical signs and symptoms of cystic fibrosis like maldigestion may not be present. The search has to be extended into "mild" mutations of the disease like D110H which was found in our case.

Tap water iontophoresis with direct current represents the therapy of choice in palmoplantar hyperhidrosis. Side effects are minor discomfort and skin irritations. Improper use may induce iontophoretic burns at sites of minor skin injuries. The aim of this study was to find ways of minimizing side effects, increasing safety standards and reducing the technical complications of tap water iontophoresis without loss of efficacy. In a blind study, 30 patients with palmar hyperhidrosis were treated with tap water iontophoresis using pulsed direct current of 4.3 kHz or 10.0 kHz. Efficacy and side effects were compared with those of the conventional direct current method as a control. Normal sweat secretion rates of palms were found after an average of ten treatment sessions with the conventional direct current method and after twelve with pulsed direct current of 4.3 or 10 kHz. Treatment with pulsed direct current of 4.3 kHz failed to inhibit palmar hyperhidrosis in two of ten patients. Occasionally, such side effects as discomfort, skin irritation, and mild electric shock occurred when direct current was applied. Using pulsed direct current subjective sensations of discomfort and skin irritation were rare (4.3 kHz) or very rare (10 kHz). Electric shock was completely prevented. Because of the minimal side effects, despite minor loss of efficacy tap water iontophoresis with pulsed direct current can be a valuable alternative treatment for palmar hyperhidrosis.

An estimation of the electrolytic losses through the skin was evaluated in a run over a distance of 10 km on a 400 m Tartan-trac. Na, K, Ca, and Mg excreted by the skin were collected in standardised clothing and thereafter washed out. The remaining electrolytes on the skin were collected by washing the body with deionized water. In addition, the concentrations of hormones and metabolites in blood before and after the race were ascertained. Mean ambiant temperature and relative humidity amounted to 21 degrees C and 35%, respectively. The mean performance was 40.5 min. and the average body weight loss was 1.45 kg. The only significant changes in the serum concentrations were the increases of free fatty acids and glycerol. This can be explained, together with a slight increase of glucose and a decrease of insulin, by a higher sympathoadrenergic activity. In the mean 20 mg calcium, 5 mg magnesium, 200 mg potassium, and 800 mg sodium were lost by the skin per kg body weight loss. Those amounts compared well with corresponding data found in literature. The described method can be proposed for further experiments.

An estimation of the electrolytic losses through the skin was evaluated in a run over a distance of 10 km on a 400 m track. Na, K, Ca and Mg excreted by the skin were collected in standardised clothes and thereafter washed out. The remaining electrolytes on the skin were collected by washing the body with deionized water. In addition the concentrations of hormones and metabolites in blood before and after the race were measured. Mean ambient temperature and relative humidity amounted to 21 degrees C and 35%, respectively. The mean performance was 40.5 min. and the average body weight loss was 1.45 kg (1.95% of body weight). The only significant changes in the serum concentrations were the increases of free fatty acids and glycerol. This can be explained, together with a slight increase of glucose and a decrease of insulin, by a higher sympathoadrenergic activity. In the mean 20 mg calcium, 5 mg magnesium, 200 mg potassium and 800 mg sodium were lost by the skin per kg body weight loss. These values compared well with corresponding data found in literature. The described method can therefore be proposed for further experiments.

The presence of cocaine, morphine and methadone in sweat samples obtained after stimulation of the eccrine sweat glands, from drugs users after six drugs-free days, was investigated. The stimulation of the sweat elimination was proved using pilocarpine-iontophoresis every hour for 7 hours. The drugs concentrations were determined by radioimmunoassay. Consequently, the values measured represent the sum of the drug and its metabolites. Measurable levels of cocaine, morphine and methadone were obtained after the third stimulation of the glands.

During heavy exercise horses can increase oxygen uptake compared to resting conditions considerably more than man. Processes involved like respiration, heart size, cardiac output, oxygen transport capacity of the blood and oxygen release in the capillaries are discussed. Besides these advantages in the aerobic metabolism conditions for the anaerobic metabolism are also more advantageous in horses than in man. The portion of fast contracting muscle fibers with little fatigue-resistance and also some of the enzymes required for the anaerobic metabolism are higher in horses.

Although active chlorine compounds have been used for more than 140 years (Semmelweis, 1848) as a skin disinfectant the phenomenon of the "chlorine covers" not earlier than 1988 has been described for the first time (Hyg. + Med. 13 (1988) 157). It deals with a chemical alteration of the uppermost skin layer which comes apparent in an oxydizing action against aqueous iodide. Its origin is chlorine covalently bound in the form of N-Cl functions to the protein matrix of the horny skin. Since the chlorine covers exhibit a persistant disinfecting activity which might be important for practice, the factors influencing their strength have been established. The most important are: the kind of the chlorine system, the concentration (oxydation capacity), pH, temperature and the volume of the used solution, the time of action, the application technique and the state of the skin. Variations of the latter can be observed at different skin areas of one and the same person as well as at the same areas of different persons, and result in differences of the cover strength up to 100%. The stability on dry skin is very good, showing a decomposition rate of approximately 1.2% per hour. However on skin surfaces moistened by sweat (e.g. hands covered by surgeons gloves) the chlorine cover is disingrated much more faster (decomposition rate: 40-50% per hour). Washing with soap as well as the action of alcohols cause virtually no decrease in the cover strength, while wetting by solutions of reducing agents (e.g. thiosulfate, cysteine, iodide) provokes a fast decomposition suitable for removing the chlorine covers.(ABSTRACT TRUNCATED AT 250 WORDS)

In 43 children and adolescents (30 healthy and 13 CF patients) the Quantitative Pilocarpine Iontophoresis Test (QPIT) was compared with the ion-specific electrode. We found an excellent correlation between these two methods (r = 0.93) and no false positive or false negative results. With both procedures the total chloride-ion-concentration in sweat of healthy subjects and patients with CF differed significantly (p less than 0.001). Both, the original Gibson and Cooke-sweat-test (QPIT) and the assessment of chloride with the sensitive electrode are highly reliable procedures for confirming the diagnosis of CF.

This study investigates the presence of drugs in apocrine sweat. The samples were collected from the axillary perspiration of 25 hs. The determination of the drugs was performed by radioimmunoassay. The concentrations measured represent the sum of drugs and there metabolites. Measurable drugs concentrations of THC, benzodiazepine, cocaine, barbiturate, morphine, methadone and cotinine were found in all samples. The presence of the drugs in the sweat was manifested also by GC/MS.

The high concentration of Na and Cl in the sweat of patients with Cystic Fibrosis is directly due to the primary pathogenetic defect, the reduced transport of Cl-Ions through cell membranes of exocrine glands. The most reliable method for the sweat test is by Gibson and Cooke. In good correlation are the results of the Wescor Macroduct System. This method is easier to perform, but needs special instruments.

The results of the investigations to reference values for Na(+)- and Cl(-)-concentrations in sweat of adults are as follows: The normal range of electrolyte values in sweat in adults is up to 70 mmol/l (Na+) and 55 mmol/l (Cl-) respectively in infants (40 mmol/l for Na+ and Cl-). There are no sex and age differences. Adults with chronical bronchitis and bronchiectasis don't show different results in comparison with healthy persons. Adults with cystic fibrosis have significantly increased sweat electrolyte concentrations (90-120 mmol/l). Variations in the electrolyte values of a day or of a month are important and much higher than the analytical one.

The presence of methadone was investigated in 24-h perspiration samples obtained from patients receiving daily maintenance doses of the drug. The samples were collected from the axillary region. The concentrations measured by radioimmunoassay ranged from 8.3 ng/cm2 per 24 h up to 25.3 ng/cm2 per 24 h. The concentrations determined by RIA are the sum of methadone and its metabolites. The presence of the drug in the perspiration samples were also revealed by GC/MS.

A positive sweat test is still the principal diagnostic feature of cystic fibrosis. Repeated tests are mandatory in positive, borderline and any suspect cases. Pilocarpine iontophoresis and quantitative evaluation of sodium and/or chloride are the only acceptable test methods. Every effort must be made to avoid contamination of the sweat sample. Diagnostic values are more than 50 mequ/l for sodium and more than 60 mequ/l for chloride.

The human horny layer contains insoluble arginase, which is tied to keratinic cells, with an activity of about 1 mU/cm2 skinsurface. About 50 p.c. of arginase is inhibited by water soluble substances of horny layer or by using soaps and syndets. By washing the skinsurface with water the activity increases. Presumable the horny layer arginase has a function in the urea production of sweat.

The concentration of gamma-glutamyl transpeptidase was determined in eccrine, thermal sweat of 56 healthy men (ages 20--60 years) and 48 healthy women (ages 17--55 years). Samples of sweat were collected from the chest and back.The concentrations of gamma-glutamyl transpeptidase showed great individual variations. The sex specific comparison of the concentrations of gamma-glutamyl transpeptidase revealed that the women excreted in the sweat from the chest and the back nearly the double amount of this enzyme. The gamma-glutamyl transpeptidase concentrations determined in the sweat from the chest of the examined men and women were three times higher as compared with those excreted simultaneous from the back of the same persons. In the group of the women was this differences statistically significant.