[Idiopathic facial hemiatrophy with generalized myopathy].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to U Runne.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Although herpes zoster is a viral infection with spontaneous healing, the skin eruption is painful and there may be serious complications. Cytarabine was administered to 91 patients with the disease. It was given by quick infusion or intravenously twice daily for five days. An open, uncontrolled study of 61 patients suggested that the drug was beneficial without serious side effects. But in a double-blind randomised study of 30 patients there was no significant effect on the course of the disease. The mean healing time for skin blisters in segmental uncomplicated zoster was 8.4 days with cytarabine and 6.5 days with a placebo.
There is a clear-cut correlation between the clinical course of an herpes zoster and the quality of immune response. A varioloid generalized herpes zoster accompanied by zoster-pneumonia developed in a forty years old female patient with metastasizing breast cancer treated with x-ray and cytostatic drugs. The particularly serious course of this herpes zoster was brought about by combined immune deficiency, consisting of low level of circulating T-lymphocytes (5/mm3) and for a long time lacking rise of complement fixing antibody titer. Under these conditions an enhanced replication and massive haematogenous spread of varicella-zoster virus took place. The increased contagiosity of this case is indicated by a varicella infection in the patient's two children, appearing after a regular incubation period. Patients with cellular or combined immune deficiency are potentially exposed to danger of herpes zoster and may serve as a source of infection for susceptible persons.
Electron microscopic investigations in 7 patients with different dermatoses or skin tumours containing amyloid showed that amyloid is synthesized in the cytoplasm of dermal cells. In 3 cases of localized primary amyloidosis of the skin highly active cells were demonstrated, showing grossly dilated cisternae of rough endoplasmic reticulum, resembling fibroblasts. Their intracellular product seemed amorphous, later filamentous, and was then released into the extracellular space. Extracellular aggregations of typical amyloid filaments were found partially surrounded by thin cytoplasmic remnants of the cells producing them. Subclinical amounts of amyloid found in porokeratosis of Mibelli, in superficial basal-cell carcinoma, in senile skin, and in clinically normal skin of a patient with malignant melanoma showed the same characteristics. Other cell types such as plasma cells and mast cells were well preserved and seemed stimulated; however, no amyloid precursors were found in their cytoplasm and no release was seen. We, therefore, conclude that dermal amyloid is generally produced by falsely programmed fibroblasts.
Six patients with facial hemiatrophy (H.f) were thoroughly examined by clinical and laboratory investigations. Two were found to have idiopathic and four facial hemiatrophy due to different types of localized scleroderma. In all cases a generalized myopathy was present, demonstrated by electromyographical, histological, and biochemical means. In none of these cases a hereditary or neurological cause for the facial hemiatrophy was found. However, in two cases autoantibodies against nuclei and muscle were repeatedly obtained. No prolongation of sensory or pain chronaxy occurred in either the patients with sclerodermal or idiopathic facial hemiatrophy. These observations suggest that facial hemiatrophy can originate (a) in a localized scleroderma in the involved part of the face, (b) in an ipsilateral "sclérodermie en coup de sabre", or (c) in the coexistence of both. There is a close pathological and physiological correlation between idiopathic and sclerodermal facial hemiatrophy. In both forms of facial hemiatrophy the disease involves skeletal muscle tissue systematically. This myopathy is similar to that of progressive scleroderma, far exceeding the limited muscular involvement of localized scleroderma. Sclerodermal facial hemiatrophy can be associated with autoimmune phenomena.
Nodular lesions of chronic prurigo correspond to highly sensitive pruritic areas of neural proliferation. This has been shown by light and electron microscopic studies in four patients. Axons and Schwann cells were participating in this process. Their ultrastructure appeared mostly normal; however, some disorganization, axonal swelling, and dystrophy were also observed. At places, newly formed nervous tissue imitated neuromas, surrounded by mononuclear cells and a peculiar cell type which we called "spider cell." The noninvolved skin near the nodules showed no alterations. Occlusion with elastic bandages for four weeks betters the clinical and histological picture. According to these findings, it seems that the neural proliferation in prurigo is a secondary phenomenon due to chronic traumatization by scratching. We also feel that the extreme pruritus in chronic lesions is related to the increased number of dermal nerves.
Thyroid carcinomas rarely metastasize to the skin. In three patients skin metastases following follicular, papillo-follicular and medullary carcinomas were seen. In two cases the metastatic spread was haematogenous, in a third patient with a retrosternal thyroid carcinoma there was massive spread through the anterior thoracic wall. The diagnosis was made on histological invesitgation of the skin lesions and after total body scintigraphy with 131I. In one seemingly-healthy patient the histological investigation of skin nodule first led to the diagnosis of thyroid carcinoma. If skin metastases occur several years after the discovery of a visceral tumour the possibility of a second primary tumour should be considered. The results of combined treatment with with surgery, radio-therapy, (radioiodine and tele-cobalt) and hormone treatment show that skin metastases in thyroid carcinoma do not necessarily have a hopeless prognosis.
Explore the source record for details and available documents.
Lanthanum has been used as a tracer for the intercellular space and the surface coats in different epithelia including human epidermis, however, lanthanum compounds may or may not penetrate into skin specimes and reaction product may or may not be present in the intercellular space. After treatment with alcian blue prior exposure to lanthanum the trilaminar plasma membrane was more regularly stained and a thin coat of reaction product corresponding to superficial glycoconjugates outlined the cell surface and filled the narrow intercellular space in normal epidermis. In psoriatic epidermis the quantity of superficial membrane-bound glycoconjugates was rather reduced, their distribution and compartmentalization was profoundly changed and the outer leaflet of the trilaminar plasma membrane was not always clearly seen, having a "moth-eaten appearance" in some places. These findings show once more that alterations of the cell membranes and their surface architecture are present in psoriasis. They may play a major role in the pathogenesis of the disease, influencing the activities of membrane-bound enzymes and the flow of the epidermal growth control mechanism. The glycocalyx may serve in this mechanism as an accumulator of energy rich compounds or as a receptor site of membrane-bound ezymes. According to our views it represents a "ground substance" of epidermal origin, which controls the social behaviour of the keratinocytes in tissue.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
A marked cutaneous axonal dystrophy has been observed electronmicroscopically for the first time in the skin of three patients: (a) lesion of pityriasis lichenoides chronica in a patient with bronchogenic carcinoma, (b) non involved skin of a patient with malignant melanoma and (c) non involved skin of a patient with gout and retinal damage after prolonged use of chloroquine. The affected myelinated and non myelinated axons showed distinct alterations of mitochondria and multiple osmiophilic lamellated bodies (LK). These changes were interpreted as a (poly-)neuropathy, due to the influence of toxic systemic agents, such as malignant tumor and abuse of drugs. Chloroquine is known to induce neural damage. Moreover, some other compounds (ergotamine, ethaverine, analgetic preparations) may also be responsible for the drug-induced axonal dystrophy described in this study.