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B Rzany

Publications and source records attributed to B Rzany.

33 records · Page 2Linked to original sources

An international collaborative case-control study of severe cutaneous adverse reactions (SCAR). Design and methods.

A multicenter international case-control study has been designed to elucidate the etiology of Stevens-Johnson syndrome and toxic epidermal necrolysis (TEN). Although these diseases occur rarely, the morbidity is high and the mortality for TEN is of the order of 30%. These serious dermatologic conditions have often been linked to exposure to drugs. Infective and autoimmune diseases, as well as other non-drug risk factors, have also been postulated to be of importance in increasing the risk. The design and methods are described, with particular attention to the unique challenges for an epidemiologic study of these conditions.

Case-Control Studies↗

Low N-acetylating capacity in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis.

Low constitutive N-acetylating capacity has been implicated as a predisposing factor for the development of adverse reactions to certain drugs. This prompted us to investigate whether the N-acetylating capacity of patients with serious cutaneous adverse reactions, i.e., Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) differed from that of healthy control subjects. N-acetylating activity was measured in hair root cells by preparing a homogenate from freshly extracted hair roots and assessing acetyl-CoA-dependent N-acetylation by RP-HPLC using 2-aminofluorene as a substrate. Samples were obtained from hospitalized patients suffering from acute SJS and TEN or from healthy controls. All patients with SJS and TEN were found to have a low N-acetylating capacity (0.85 nmol/mg/min compared to 2.21 nmol/mg/min in controls, p < 0.05). Based on these findings, a low constitutive N-acetylating capacity may be one of the predisposing factors for the development of serious cutaneous adverse reactions to drugs that require N-acetylation in these patients.

Acetylation↗

Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme.

BACKGROUND AND DESIGN: To conduct a prospective case-control study about causative factors of severe bullous erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis, we needed to define criteria for classifying the cases and standardize the collection of data so that cases could be reliably diagnosed according to this classification. Based on review of case histories and photographs of patients, a group of experts proposed a classification based on the pattern of erythema multiforme-like lesions (categorized as typical targets, raised or flat atypical targets, and purpuric macules) and on the extent of epidermal detachment. An atlas illustrating this classification that included photographs and schematic drawings was developed. We compared the evaluations of 28 cases by four nonphysicians relying on the atlas with the evaluations of the same cases by five experts not using the atlas to determine the usefulness of this atlas for classifying cases according to our nosologic schema. RESULTS: The following consensus classification in five categories was proposed: bullous erythema multiforme, detachment below 10% of the body surface area plus localized "typical targets" or "raised atypical targets"; Stevens-Johnson syndrome, detachment below 10% of the body surface area plus widespread erythematous or purpuric macules or flat atypical targets; overlap Stevens-Johnson syndrome-toxic epidermal necrolysis, detachment between 10% and 30% of the body surface area plus widespread purpuric macules or flat atypical targets; toxic epidermal necrolysis with spots, detachment above 30% of the body surface area plus widespread purpuric macules or flat atypical targets; and toxic epidermal necrolysis without spots, detachment above 10% of the body surface area with large epidermal sheets and without any purpuric macule or target. Using the atlas, the nonexperts showed excellent agreement with the experts. CONCLUSION: This study suggests that an illustrated atlas is a useful tool for standardizing the diagnosis of acute severe bullous disorders that are attributed to drugs or infectious agents. Whether the five categories proposed represent distinct etiopathologic entities will require further epidemiologic and laboratory investigations.

Erythema Multiforme↗

Toxic epidermal necrolysis and Stevens-Johnson syndrome. An epidemiologic study from West Germany.

Little work has been carried out on the epidemiology of the two serious skin reactions--toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS). We collected details of all the hospitalized cases of TEN and SJS in the Federal Republic of Germany for the years 1981 through 1985 inclusive. Inquiries by telephone, letter, and personal visits produced an overall response of 91%; 259 cases of TEN and 315 cases of SJS were identified. From these data, we were able to calculate an overall annual risk of 0.93 and 1.1 per million for TEN and SJS, respectively. The average age group was higher for TEN (63 years) than for SJS (25 years). Women are markedly more at risk for TEN in the ratio of 2:1, these figures being reversed for SJS. The mortality was 34% (87/259) for TEN and only 1% (2/315) for SJS. An association with previous medication defined as "definite, probable, possible" could be established for 89% of cases of TEN and 54% of cases of SJS. The drugs most commonly involved were antibiotics (TEN, 40%; SJS, 34%), followed by the analgesics (TEN, 23%; SJS, 33%). As with the drug groups, the incidences being based on the defined daily doses, were high for sulfonamides, beta-lactam antibiotics, and some nonsteroidal anti-inflammatory drugs.

Adult↗

Clinical characteristics of patients with repeated systemic reactions during specific immunotherapy with hymenoptera venoms. A retrospective study.

In general, specific immunotherapy with hymenoptera venoms can be considered as safe, but occasionally there are patients who cannot reach the maintenance dose due to repeated systemic reactions (RSR) or who suffer from RSR during maintenance therapy. In a multicenter retrospective study comprising seven departments in Germany, Austria and Switzerland 23 patients with RSR were reported from approximately 3000 patients treated with hymenoptera venoms (bee and wasp venom to approximately equivalent frequency). From these, 22 were allergic to bee venom and only one to vespid venom. In general the clinical symptoms of RSR were milder than the initial reaction. But 4/23 (18%) exhibited cardiovascular reactions up to full shock. Neither anamnestic details, reactivity in skin tests or in vitro tests revealed a special pattern of patients with RSR. In some patients, however, an extremely high reactivity in the skin test was found and may indicate the possibility of further RSR.

Adolescent↗

Toxic epidermal necrolysis in patients receiving glucocorticosteroids.

Despite the lack of evidence of an immunologically mediated mechanism for Toxic Epidermal Necrolysis (TEN), glucocorticosteroids are frequently used for the treatment of patients afflicted with TEN. We investigated the data of 216 patients with TEN for therapy with glucocorticosteroids prior to the beginning of TEN. The data had been collected between 1984 and 1985 during the epidemiological study on severe skin reactions in West-Germany spanning the years 1981-85. 11/216 (5%) patients could be traced, who had been treated with glucocorticosteroids for at least a week prior to the first dermatological sign of TEN. Glucocorticosteroids had been administered predominantly for neurological or neurosurgical conditions. The period of treatment ranged from 1 week to several months. Doses ranging between 7.5 and 325 mg prednisolone/per day were found. There were no major differences between patients with or without steroid therapy with regard to sex, age and lethality. The observation of TEN in patients receiving therapy with glucocorticosteroids raises questions about the usefulness of treating patients with TEN with glucocorticosteroids.

Adult↗

[Cutaneous leishmaniasis: treatment with cryotherapy and intramuscular injections of meglumine antimonate (glucantime)].

A 37-year-old female patient with cutaneous leishmaniasis of the helix of the left ear is reported. The diagnosis was based on her past history, clinical appearance and histological examination (detection of Leishman-Donovan bodies). Initial cryotherapy failed to cure the lesions completely. Treatment with intramuscular injections of meglumine antimonate (Glucantime) was successful. The discussion focuses on the pathophysiology, diagnosis and therapy.

Adult↗

[Specific IgE antibodies to cow's milk and individual cow's milk proteins in children with suspected allergy to cow's milk].

Specific IgE antibodies against whole-milk-antigen mixture and against casein, alpha-lactalbumin and beta-lactoglobulin were determined using the radioallergosorbent test (RAST) in 125 sera of children with an initial diagnosis of suspected cow milk allergy. The results of the RAST were correlated with the clinical diagnosis. No specific IgE antibodies could be demonstrated in 98 children. Of these, 20 (20%) showed clinically intolerance to cow milk presumably on the basis of other immunological mechanisms. Specific IgE antibodies could be demonstrated in the sera of 27 children. Of these, 16 (60%) actually showed allergy to cow milk. A statistically significant association with chi 2 test could be established between positive RAST and clinical diagnosis (p = 0.001).

Adolescent↗

Interferential current is effective in palmar psoriaris: an open prospective trial.

Interferential current (IFC) has been shown to improve psoriasis in a small case series. So far no formal clinical trial had been conducted. As IFC is associated with slight prickling sensations a blinded study design was not feasible. Therefore, an open type prospective study was conducted with the assumption of 18% spontaneous remission rate. A response rate of 50% or less was judged as indicating no effect (hypothetical control), while 80% or more was considered as success (alternative hypothesis). In this "quasi-controlled" study 12 patients with therapy resistant palmar psoriasis received local treatment with IFC during a 12 week period. Treatment was performed at low current density in two daily sessions, each of 6 minutes duration. Erythema, scaling, induration, fissures and pustules were recorded in separate scores every 4 weeks. Response of a patient was judged positive when the total score of these criteria was reduced at least by two points at the end of treatment. After 12 weeks of treatment, 11 of 12 patients were cured or showed marked remission with the median overall score reduced by 4 points. An interim analysis was performed in order to decide whether the results had already reached significance (a < 0.05). The analysis revealed a statistically significant response rate of 90% (95% confidence interval 62-99%). These results are highly encouraging and should focus attention on this new therapy modality, which, in contrast to other treatments is not associated with side effects and discomfort.

Adult↗