Biomedical subjects
Publications and source records attributed to E Hoting.
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Trichothiodystrophy leading to generalized trichorrhexis nodosa-like hair changes with abnormal hair breakage is described in a 4-year-old girl. A marked deficiency of sulphur and the sulphur-containing amino acid, cystine, was detected in the biochemical analysis of the hair. Further investigation of the hair showed the morphological criteria of trichothiodystrophy. Commonly related symptoms, such as mental retardation, ichthyosis and increased sensitivity to sunlight, were not present in our patient.
The properties of diphencyprone (DPC), its possible mechanism of action in the therapy of alopecia areata (AA), and its side-effects are summarized. The results of our own study of treatment in 45 patients with AA, with a mean treatment duration of 72 (15-146) weeks, are presented, and compared with the results of previous studies. We suggest that, in future, therapy protocols should define entry criteria and therapy results more precisely, and include follow-up assessments to establish the length of remission. In view of the low response rates and short lengths of remission, the indications for DPC therapy should be set more rigorously.
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Patients suffering from atopic dermatitis show increased phosphodiesterase activity in leucocytes. As a result, the level of intracellular c-AMP is reduced, which can produce a number of metabolic dysfunctions: We found strong evidence that an enhanced release of several inflammation mediators can favor itching, which in turn promotes the development of eczema, since it induces reflectory scratching. In order to solve the question whether papaverine as a phosphodiesterase inhibitor might offer a new approach in the systemic therapy of itching, we performed a placebo-controlled study on 51 patients with atopic dermatitis, who were orally treated with papaverin-HCl over 8 weeks. However, we did not observe any improvement of itching or eczema during treatment. Some other recently developed phosphodiesterase inhibitors are discussed.
Five patients with refractory advanced cutaneous T-cell lymphoma (CTCL) (Stage IIB, two; Stage III, one; Stage IVA, two) and one patient with a refractory Stage IB CTCL (two females, four males, median age 58 years) were treated with a new polyaromatic retinoid, arotinoid-ethylester (Ro 13-6298), which has a much higher antiproliferative activity than other known retinoids. There was an objective clinical response in three of six cases [complete remission (CR) = 1, partial remission (PR) = 2]. One patient is in CR since 102 weeks currently. Mean duration of PR was 43 weeks. Two patients were withdrawn from treatment after 4 weeks because of disease progression. Two patients, one of them in PR, had to be retired from further treatment due to toxic side effects. Main side effects were mucocutaneous dryness, skin atrophy, and skin vulnerability. Skin biopsies were performed on four patients 4 weeks after the start of treatment. The epidermis and the subepidermal grenz zone were found to be clear of mononuclear clear cell infiltration in three patients. Although the number of patients is small, the results obtained suggest that arotinoid-ethylester offers a promising approach to the treatment of CTCL.
175 patients with severe psoriasis of different types were treated with 10, 25, or 50 mg acitretin and compared with patients receiving 50 mg etretinate over a period of 8 weeks in a randomized, double-blind multicenter study in the Federal Republic of Germany. Plasma concentrations of etretinate and its metabolite acitretin were measured during therapy and also 3 weeks after cessation of treatment. After 4 weeks of treatment, a trend toward clinical improvement was shown in all groups with increasing dosage. Those groups receiving the lower acitretin doses (i.e., 10 and 25 mg/day) had more dropouts than the groups taking 50 mg acitretin or 50 mg etretinate. Complete remissions before the end of therapy occurred only among those receiving higher doses. Enlargement of psoriatic lesions, however, could be observed during treatment with both retinoids, despite improvement of other parameters, as measured by psoriasis area and severity index (PASI) and psoriasis severity index (PSI). After 8 weeks, a significant improvement was calculated by the PASI score and by a newly defined, corrected PASI score for all four dose regimens compared with baseline levels. A greater than 50% PSI score improvement was seen in 50% of patients treated with 10 mg acitretin, 40.5% with 25 mg acitretin, 53.8% with 50 mg acitretin, and 61.1% with 50 mg etretinate. No statistical differences were found among these groups at any time during the 8-week period. No new or unexpected side effects occurred during acitretin treatment. Moreover, cholesterol levels did not significantly change. Three weeks after cessation of drug administration, the plasma concentrations of acitretin were below the sensitivity level of the assay, whereas etretinate was still quantifiable. It is interesting that acitretin plasma concentrations during therapy with 50 mg acitretin were markedly lower (means = 18 ng/ml) than were acitretin levels during treatment with 50 mg etretinate (means = 36 ng/ml).
Papular acrodermatitis (Gianotti-Crosti syndrome) was seen in a six-year-old girl. The disease was marked by the characteristic triad of a papular-vesicular rash, lymphadenopathy and liver damage. Serological findings suggest an infection with Epstein-Barr virus as the causative factor. In such cases hepatitis-B induced papular eruptive acrodermatitis should be considered in differential diagnosis.
A multicenter trial of isotretinoin in severe papulopustular rosacea in adult patients was undertaken to evaluate the efficacy and safety of this treatment. A 20-week course of therapy was instituted in 92 patients from 11 dermatology departments. At the end of the study period, isotretinoin was found to be highly effective in the clearing of refractory rosacea lesions.
On the basis of two case-reports it is concluded that the aromatic derivative of retinoic acid, etretinate, is a promising drug in the treatment of pachyonychia congenita.
Case report on a 50-year-old man suffering from discoid lupus erythematosus since about 20 years. The development of a tumor of the left lower leg within a l. e. area, which turned out to be a squamous-cell carcinoma, had been noticed since two years. One year after tumor excision our patient died of multiple metastases. This case demonstrates the rare formation of a squamous-cell-carcinoma "in erythematode ".
Carprofen, a non-steroidal anti-inflammatory drug of the arylpropionic acid derivative type, can provoke both phototoxic and--as in the reported case--photoallergic reactions.
Statistical evaluation of epicutaneous tests performed in 5348 patients during the years 1976 to 1980 revealed positive reactions towards topical steroids in nine cases. Three patients showed an allergy due to Dermoxin (Dermovate). Further tests with the single components of the steroidal cream disclosed a specific hypersensitivity towards clobetasol-17-propionate in two cases, towards the preservative 4-chlor-m-kresol in the third case. Cross-reactions to other fluorinated corticosteroids were not obtained. Experimental sensitisation with clobetasol-17-propionate in guinea pigs remained negative although different methods have been employed.
The diagnostic procedure in gonorrhoea always starts with staining methods that the physician at first has to rely on in spite of their limited evidence, and that may justify specific treatment. Only culture, oxydase reaction, and biochemical differentiation (e.g. carbohydrate degradation test) can provide exact identification of pathogenic species of Neisseria. Further techniques can answer for antibiotic resistance and penicillinase production. Serological methods are employed with chronic or complicated gonococcal infections as well as with epidemiological investigations.
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Retinoids possess regulatory influences on growth and differentiation of epithelial tissues. They induce a population of keratinozytes with normal pattern of differentiation, they have antiproliferative properties, and they show antineoplastic effects by inhibition of malignant transformation of cells in vitro. Also the dermis undergoes distinct alterations under oral administration of retinoids. By stimulating T-lymphocytes and by inhibition of neutrophil migration retinoids seem to develop immunmodulating and antiinflammatory effects. The aromatic retinoid Etretinate is therapeutically used in severe forms of psoriasis and in various genodermatoses with disorders of keratinization as for example ichthyosis, dyskeratosis follicularis Darier, and pityriasis rubra pilaris.
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Two patients treated topically against dermatophytes with a naftifine containing antifungal became allergic to the efficacious component naftifine. One of them also reacted to benzyl alcohol. Sensitization experiments in guinea pigs corroborated the suspicion that this allylamine is a contact sensitizer. To date, only few cases of allergic reactions to naftifine have been reported.