[Pemphigus vegetans with disorders in cellular immunity].
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Biomedical subjects
Publications and source records attributed to D Djawari.
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Report on the application of a dinitrochlorobenzene ointment of 61 postoperative melanoma patients exhibiting clinical stages I and II. After contact sensitization the erythemogenic threshold concentrations of DNCB were mostly found in the range of 0,05% and 0,1%. Patients with reactions at low concentrations of 0,01% and 0,05% DNCB were in the mean 8 years younger than those with reactions at 0,1% and 0,5%, but no connection to different stages of malignant melanoma could be evaluated. 3 melanoma patients suffering from skin metastases were treated by epifocal DNCB-application. One of them became clinically tumor free since more than 1 year, whereas the two other exhibiting multicentric and/or profound tumor growth did not respond. In a 82-year-old wife a superficial lentigo maligna melanoma disappeared by DNCB-application. In none of the 61 cases we observed a "tumor enhancement" after immunoprophylaxis or adjuvant immunotherapy with DNCB. The DNCB-method in malignant melanoma is yet in the experimental stage and is not recommended for general use in practice.
In 5 patients (3 children, 2 adults) suffering from familial or non-familial chronic muco-cutaneous Candidosis (CMCC) combined with cellular immune deficiencies of different extent, an immunostimulating therapy regimen with levamisole was performed over 6 months. Post-treatment clinical and immunological re-examinations offered a favourable effect on the cellular immune system in all patients. As to the general health condition, in the children all clinical symptoms of CMCC clearly improved or disappeared completely, whereas in both adult patients no satisfactory clinical effect of levamisole therapy could be observed so far.
Typical signs and symptoms of type III allergy occurred in a 55-year-old diabetic during diabetes and diuretic treatment with glisoxepid, glibenclamide, furosemide and probenecid. Symptoms of this type of allergy unintentionally recurred with every subsequent therapeutic administration of each one of these drugs. The possibility of a cross allergy between sulphonamide diuretics, chemotherapeutic sulphonamides and sulphonyl-urea compounds has only rarely been described.
In five patients with either familial or non-familial type of chronic mucocutaneous candidosis some properties of phagocytic function of the polymorphonuclear leucocytes (PMNL) have been studied in vitro. In each of the patients there were found: a) a decreased chemotactic activity of PMNL, b) a weakness of intake and of intracellular destruction of Candida albicans cells by PMNL, c) an impairment of phagocytosis and intracellular killing of Candida albicans as well of Staphylococcus aureus by PMNL. The rate of phagocytosis of heat-inactivated Candida albicans cells by PMNL was normal in each case. In the serum of two patients a phagocytosis inhibiting factor is supposed to exist. In PMNL of 3 patients a defective activity of NADH-dependent oxidase was found. The occurrence of hereditary CMCC in a father and his two daughters points to an autosomal dominant trait, whereas in most cases of familiar CMCC hitherto described an autosomal recessive mode of transmission was found.
The function of microphages has been studied in two patients with chronic pyoderma vegetans by in vitro determination of phagocytosis as well as chemotaxis. The results showed a striking decrease in the chemotactic activity of the neutrophil granulocytes, a reduced phagocytosis of Candida albicans and Staphylococcus aureus, and a weakness of the intracellular killing of these microorganisms. However, the NADH-dependent oxidase activity appeared to be intact. No defect was found in the specific cellular or humoral immune system in either patient.
The chemotactic microphage function in combating and eliminating microorganisms is one of the most important features of the cellular immune system. Using a modified Boyden method we studied in vitro the chemotactic activity of the granulocytes in 5 patients suffering from chronic muco-cutaneous candidosis (CMCC), 3 of the familial and 2 of the non-familial type. To obtain quantitative results comparable to those of a control group, we investigated the granulocytes of 55 clinically and immunologically healthy persons by the same way. So we found, in comparison with the results of the controls, a striking reduction of the chemotactic granulocyte activity in all CMCC patients. This impairment of chemotaxis is apparently due to an inborn defect of the microphages. It is pointed out, however, that a non-specific stimulation of the immune system might be a promising way in the treatment of patients affected by CMCC.
The granulocytes which are distributed by blood circulation close to the extern and intern body surfaces as well as in all organs protect the organism from microbial perils by phagocytosis and intracellular killing of bacteria and fungi so constituting a very important component of the granulocytic functions seriously impairs the host resistance and may entail a state of persisting infectious disease. As chronic mucocutaneous candidosis (CMCC) represents such a persistent generalized infection, we studied in vitro several functional activities of the granulocytes of 5 patients suffering from CMCC. 2 of them presented a non-familiar type of CMCC, the remaining 3 patients (father and 2 daughters) were subject to the hereditary type of CMCC. For comparison, by the same way we investigated the granulocytic functions of 51 clinically and immunologically healthy adult persons. Each of our 5 patients exhibited a reduced ability of the granulocytes to phagocytize and kill Candida albicans. In the CMCC family, the father had a marked deficiency of the oxidase activity of the granulocytes whereas in his daughters, the oxidase deficiency proved to be of a minor grade. In the sera of both daughters a phagocytosis inhibiting factor could be assumed to exist in addition to the granulocytic abnormalities. When heat-inactivated Candida albicans cells, however, were used for experiments, the granulocytes of each patient were able to phagocytize the germs at the same rate as did the granulocytes taken from the controls. With regard to alterations of the T cell function previously reported in CMCC, in all patients we also could demonstrate various symptoms of a T cell-dependent immunodeficiency. The results of the present in vitro-experiments furnish good evidence of a state of fundamental deficiency of the microphages in patients with CMCC, which may be the dominating cellular factor in the aetiopathogenesis of CMCC.
There is a variety of immunologic and etiologic phenomena in Lupus Erythematosus Disseminatus (LED) sive Visceralis particularly when induced by drugs ("Pseudo-LE-Syndrom"). In this report a patient (age 22 y.) is presented suffering from LED in whom the disease exacerbated under treatment with Ethambutol. Various drugs known to either cause a "pseudo-LE-Syndrom" or to induce or aggravate a LED have been compiled in this paper and listed in tablets; Ethambutol apparently represents a new addition to the drugs listed.
The function of microphages has been studied in vitro in a 5 year-old girl with Papillon-Lefèvre syndrome. The results showed a weakness in chemotatic activity, a defect of intracellular killing of Staphylococcus aureus, and an almost normal phagocytosis but decreased intracellular killing of Candida albicans by the microphages.
The function of microphages has been studied in two patients with chronic pyoderma vegetans by in vitro determination of phagocytosis as well as chemotaxis. The results showed a striking decrease in the chemotactic activity of the neutrophil granulocytes, a reduced phagocytosis of Candida albicans and Staphylococcus aureus, and a weakness of the intracellular killing of these microorganisms. However, the NADH-dependent oxidase activity appeared to be intact. No defect was found in the specific cellular or humoral immune system in either patient.
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Specific infiltrations in myelogenous leukemia are rare and occur mostly in the course of an acute blast crisis often representing the terminal phase of the disease. A 50 year-old female patient is described who developed specific cutaneous lesions during such a blast crisis. The infiltrates consisted to a major part of eosinophilic myelocytes although the peripheral blood smear showed only relatively few mature eosinophils.