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Biomedical subjects

E Christophers

Publications and source records attributed to E Christophers.

At least 145 records · Page 8Linked to original sources

Urticarial and anaphylactoid reactions following ethanol intake.

Ingestion of ethyl alcohol may be associated with a number of adverse reactions. Apart from toxicological effects, intolerance syndromes occur, which are caused by genetic or acquired defects in alcohol metabolism and are manifest clinically as flushing. In addition to these abnormalities, rare cases of generalized urticaria and anaphylactoid reactions after ingestion of ethyl alcohol have been reported, the pathogenesis of which is still a matter of debate. We describe three patients who presented with recurrent generalized urticaria, which developed within minutes of consumption of small amounts of ethyl alcohol. Common causes of chronic recurrent urticaria were excluded by case history, physical examination and laboratory investigations, and by comprehensive allergy testing. All patients produce positive prick tests with acetic acid, and developed urticaria after oral challenge with small amounts of highly purified ethyl alcohol. The symptoms are most probably caused by an intolerance to ethyl alcohol or its metabolites, whereas an allergy sensu strictu seems unlikely.

Adult↗

Inhibitor of the release of mast cell mediators does not improve the psoriatic plaque.

The number of mast cells is increased in psoriatic lesions and this is particularly prominent in their early phase. Mediators released by mast cells may interfere with various aspects of cutaneous inflammation and epidermal proliferation. Therefore, the aim of the present investigation was to find out whether a 4-week treatment period with Tiacrilast, a highly potent inhibitor of mast cell degranulation, might have antipsoriatic potential. A total of 31 patients with plaque-type psoriasis were evaluated after treatment with a 3% Tiacrilast hydrogel and hydrogel alone, in a double-blind, placebo-controlled, within-patient comparative study. No statistically significant improvement of the Tiacrilast-treated plaques compared to the hydrogel-treated sites could be demonstrated. Therefore, the present study does not provide evidence of a potential role of mast cell degranulation in the treatment of psoriasis.

Adult↗

Diagnostic assessment of two novel proliferation-specific antigens in benign and malignant melanocytic lesions.

The aim of this study was to gain a thorough insight into the proliferative activity of benign and malignant melanocytic tumors. A total of 314 cases were examined by immunohistochemistry on paraffin-embedded material. The growth fraction was assessed by means of two monoclonal antibodies, Ki-S1 and Ki-S5, which react with two different proliferation-specific nuclear antigens. Additionally, HMB-45 was used as a marker of melanocytic activation. Statistically significant differences (P < 0.01) in the proliferation rates were found between common acquired nevi, Spitz's/Reed's nevi, primary cutaneous melanomas, and metastatic melanomas, whereas dysplastic nevi were hardly distinct from other nevi of the compound type. In melanoma, the growth fraction correlated well with the tumor stage but poorly with HMB-45 expression and mitotic count. Along with tumor progression, an increasing heterogeneity of proliferation indices was observed. Our results provide no evidence for a progression from dysplastic nevi into melanoma. They indicate that the assessment of the proliferative activity may be of considerable diagnostic help in cases of uncertain histology and that it might contribute to an alternative concept for the classification of melanocytic tumors.

Adolescent↗

Guttate and plaque psoriasis.

Guttate and plaque psoriasis are regarded as variants of one disease but present with distinct clinical and histologic appearances. This article focuses special attention on different pathways in the development and clinical course of each, with emphasis on immunologic correlations regarding HLA system linkage and therefore genotypical expression. The differences in the variants are also important in treatment regimens.

Genetic Linkage↗

[Innovative balneotherapy with reduced bath volume: foil baths].

Balneo-phototherapy, in which salt baths are followed by UV-B irradiation, has proved successful in the treatment of psoriasis. Because of practical problems, the major one of which is the large turnover of bath solution volumes, balneotherapy has so far been limited to specialized treatment centres. With the aid of a polyethylene foil the volume of bathing solutions needed can be reduced to a total of 41 per bath. Balneotherapy using small bath solution volumes for total body treatment can now be applied as an outpatient regimen for salt bath and bath-PUVA therapy. The methods for establishing balneotherapy with restricted water volumes and the advantages of bath-PUVA over conventional oral psoralen therapy are described.

Balneology↗

[Erythema necroticans migrans without glucagonoma].

Necrolytic migratory erythema is considered to be one of five well-defined paraneoplastic syndromes of the skin. An association with a pancreatic A-cell glucagonoma is commonly postulated. However, during the last years 10 cases of necrolytic migratory erythema with no evidence for glucagonoma have been published. We report on a 59-year-old woman who presented with typical clinical and histological findings of necrolytic migratory erythema. Staging and laboratory investigations gave no evidence of any neoplasia. A liver biopsy revealed chronic persisting hepatitis. Hepatocellular dysfunction was recently reported to be present concomitantly in a number of cases of necrolytic migratory erythema, suggesting a relation to the skin changes described here.

Biopsy↗

[Filiform keratosis].

A 53-year-old female patient developed disseminated, filiform keratoses up to 4 mm long on the back, arms and neck and upper thorax for the first time 10 years ago. There were no associated diseases or symptoms. Treatment with etretinate was successful. After discontinuing this therapy the patient had a relapse. With this observation we bring new arguments into the discussion on filiform keratoses.

Biopsy↗

[Multiple melanoma in xeroderma pigmentosum].

Xeroderma pigmentosum comprises a heterogeneous group of autosomal recessive hereditary diseases, which are characterized by a number of clinical characteristics and an abnormal DNA repair mechanism. Patients affected show a high frequency of mucocutaneous malignant tumors, especially squamous cell carcinomas and basal cell carcinomas. We report on a 65-year-old patient who successively developed a total of 15 malignant melanomas, 1 squamous cell carcinoma and 1 lymph node metastasis of a malignant melanoma. The clinical diagnosis of xeroderma pigmentosum was confirmed by the complementation analysis, which defined our patient as xeroderma pigmentosum of the complementation group D.

Aged↗

Of genes and antigens: the inheritance of psoriasis.

Psoriasis is one of a number of autoimmune diseases that display significant HLA associations. In particular, individuals with onset of disease prior to 40 years of age display striking associations with HLA-Cw6 and are much more likely to have a positive family for psoriasis. However, only about 10% of Cw6-positive individuals develop disease, suggesting that other genetic and/or environmental factors must be involved. Several compelling lines of epidemiologic evidence indicate that psoriasis susceptibility is inherited, albeit not in a simple monogenic fashion, and that genetic, rather than environmental, factors are primarily responsible for the variability in inheritance of psoriasis. Taken together, these observations suggest that one or more loci in addition to HLA are necessary for the development of psoriasis. The number of additional loci is likely to be small, because i) the disease is very common ii) substantial excess risk of psoriasis is observed in first degree relatives, and iii) nevoid variants of psoriasis have been reported, suggestive of somatic mutation of a single gene during development. The substantial homogeneity of the psoriatic phenotype and the clear evidence for increased HLA association and heritability in juvenile onset disease indicate that despite its complexity, psoriasis is a common disease whose etiology is amendable to elucidation through the techniques of modern molecular genetics.

Age of Onset↗

Identification of the cytokine RANTES released from platelets as an eosinophil chemotactic factor.

Selective infiltration of eosinophils suggests the presence of the factors that attract eosinophils preferentially. Selective chemotactic polypeptides for human eosinophils were purified from thrombin-stimulated platelet supernatants and this chemotaxin was identified as the chemokine RANTES. The isolation of natural RANTES and its activity for eosinophil migration is summarized here. RANTES induced eosinophil migration at nanomolar range, in both chemotactic and chemokinetic manners. Specific desensitization experiments indicated that RANTES binds to receptors different from those for C5a and PAF, well-known potent eosinophil chemotaxins.

Blood Platelets↗

The genetics of psoriasis.

BACKGROUND AND DESIGN: Psoriasis is a member of a class of common, HLA-associated conditions in which disease susceptibility appears to be heritable. However, the mode of inheritance of these diseases has been difficult to define in simple mendelian terms. Psoriasis displays one of the strongest HLA associations of this class of diseases. However, only a small fraction of those who carry the implicated HLA susceptibility alleles develop disease, and it has proven difficult to demonstrate that the HLA associations observed are due to formal genetic linkage between the disease and the HLA locus. Although the role of environmental factors in psoriasis and these other diseases cannot be denied, the participation of additional genes, not necessarily linked to HLA, has long been suspected. OBSERVATIONS: Epidemiologic and immunogenetic data are reviewed and analyzed, which demonstrate that a predisposition to psoriasis is heritable, and which implicate genes of the HLA locus as necessary but not sufficient determinants of psoriasis. Recent developments in human genome research are described, which make possible a systematic search for additional genetic determinants of psoriasis, including those unlinked to HLA. CONCLUSIONS: As one of the most common, most heritable, and most highly HLA-associated examples of this class of HLA-associated diseases, psoriasis represents an ideal target for the application of this emerging genomic technology.

Adolescent↗

[Rothmund-Thomson syndrome].

One year after a normal birth a girl developed large, flat and net-like telangiectasias and erythema with hyper- and depigmentation (poikiloderma), at first in the face and later on the limbs, sparing the trunk. There was increased light sensitivity of the affected areas. At 3 years and 8 months of age her height was diminished (3rd percentile; height 104 cm, weight 15 kg) and she had a deep nasal root and bradydactylia. These cutaneous and extracutaneous abnormalities correspond to the spectrum of Rothmund-Thomson syndrome which has a wide clinical heterogeneity. The family history for this condition was negative.

Child, Preschool↗

[Dramatic remission of a multiple metastasized melanoma caused by chemo-immunotherapy].

A 47 year old woman underwent excision of a malignant melanoma on the right shoulder. Nine years later she was attacked by intense pain in the back. Computed tomography, bone scans and immunohistochemical study of a liver biopsy specimen revealed metastases from the malignant melanoma in the liver, spleen and skeleton. Her Karnovsky score was 50%. Chemoimmunotherapy given for five cycles consisting of dacarbazine (800 mg/m2 body surface area every 4 weeks) together with ambulatory cytokine therapy (interferon alpha-2b subcutaneously, 5 million I.U. on days 1 and 3 to 7 in the first week, days 1, 3 and 5 in the second and third weeks and interleukin 2.9 million I.U. on days 1 to 5 in the second and third weeks; no treatment was given in the fourth week) raised the Karnovsky score to 90% and abolished the pain. Lactate dehydrogenase activity, originally 3372 U/l, dropped to normal. Computed tomography demonstrated definite shrinkage of the liver metastases and some regression of the splenic and bony deposits. This improvement has now been maintained for a total of eight cycles of therapy.

Antineoplastic Combined Chemotherapy Protocols↗