[Changes of the central nervous system in scleroderma (progressive systemic sclerosis)].
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Biomedical subjects
Publications and source records attributed to E Haneke.
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Urocanic acid was measured by means of high-performance liquid chromatography (HPLC) in the stratum corneum of a 3.5-year-old child suffering from hidroa vacciniformia. Compared to ten healthy children of equal age, the urocanic acid content of the stratum corneum was reduced significantly. In addition, there was an elevated level of some amino acids in the urine, particularly that of histidine and methylhistidine, which seems to be a very important finding. Urocanic acid acts as a natural sun protective agent, and the results are discussed with this in mind. It was not possible to elicit hidroa vacciniformia lesions in the patient by UV-A, as described by others.
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In a 53-year-old man with Sézary's syndrome, histology showed unique epithelial islands originating from the sweat glands, epithelioid granulomas with giant cells in lymph nodes, and abundant foam cells in the papillary layer as well as perivascularly in the upper dermis and lymph nodes. Severe cholestatic granulomatous hepatitis and hyperlipoproteinemia caused further aggravation of the disease.
A 47-year-old man suffering from colitis ulcerosa developed serious anaemia, oedema, stomatitis with burning tongue, dry skin and erosive lesions with peripheral spreading on the ankles. Histology and electron microscopy showed characteristic signs for necrolytic migratory erythema, zinc deficiency acrodermatitis, or pellagra. Decreased serum zinc level and response to oral zinc substitution finally proved the diagnosis of acquired zinc deficiency.
Treatment resistant recurrent herpes simplex persisting for years in 7 patients was treated with thymopoietin pentapeptide for 6 weeks, 50 mg subcutaneously three times per week. During treatment and follow-up of 6 weeks a pronounced reduction of frequency and duration of recurrences were observed. There was some tendency for normalisation of deranged lymphocyte and granulocyte function. The mode of action of thymopoietin pentapeptide in recurrent herpes simplex remains uncertain. An indirect effect via stimulation of production of interferon may be assumed.
Hyalinosis cutis et mucosae is a rare autosomal recessive disorder of unknown aetiology. Clinical features are hoarseness from early infancy and yellowish papules and plaques in mucous membranes and skin. Light and electron microscopy demonstrates accumulation of PAS-positive material around blood vessels and in the dermis. This substance probably derives from glycoproteins of basement membranes. Carbon dioxide laser surgery of thickened vocal cords proved to be effective for the treatment of hoarseness.
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Twenty-four patients suffering from longstanding severe recurrent herpes simplex, who had not responded to prior therapy, were treated with s.c. thymopentin injections 50 mg, three times weekly, over a period of six weeks. They were followed up at weekly intervals over this period and then six weeks later. Moreover, the longest relapse-free period observed in the year after the treatment was recorded in the investigator's documentation. Thirteen of the 14 patients with labial herpes simplex and 10 of the 13 patients with genital herpes simplex improved markedly as shown by a decrease in the relapse rate of at least 50%, shorter episodes of relapse and improvement of symptoms such as pain and itching. Fourteen of these 27 patients experienced no relapse for a period longer than four months after cessation of the therapy. No serious side-effects were observed. Laboratory examinations before, during and after thymopentin did not reveal significant alterations except for an increase in the T-helper/T-suppressor ratio. The effect of thymopentin is assumed to be due to T-helper cell activation resulting in enhanced interleukin-2 production with subsequent proliferation of cytotoxic T lymphocytes and natural killer cells which are capable of producing immune interferon.
Melanonychia striata longitudinalis is due to a focal increase in number and/or activity of melanocytes in the nail matrix, resulting in the continuous production of large amounts of melanin which are transferred to the nail cells and grow out with the nail plate. Histologically, either benign melanocytic hyperplasia, lentigo simplex, nevocellular nevus, atypical melanocytic hyperplasia or acral lentiginous melanoma may be found. Longitudinal pigmented streaks in the nails of Caucasians tend to be malignant rather than benign. Thus a biopsy for proper histological diagnosis is strongly advised. Melanonychia striata may also occur as a symptom of rare syndromes and diseases or be due to therapy. Differentiating this condition from subungual hematoma and infections with chromogenic bacteria and fungi may be difficult.
We report the first German case of Iso-Kikuchi syndrome, which is characterized by a congenital onychodysplasia commonly involving the index fingers, but not infrequently also the neighbouring fingers, and typical bony alterations of the corresponding terminal phalanges. This is also the first report of associated great toe anomalies.
Ketoconazole, a new orally active broad-spectrum antimycotic, is usually well tolerated. Liver changes have been observed with an incidence rate below 1:10 000; they are idiosyncratic reactions independent from duration of treatment or dosage. Ketoconazole given in a dosage of 400 mg daily or more may inhibit testosterone biosynthesis.
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Five patients suffering from recurrent oral aphthae and one patient with Behçet's disease were treated with thymopoetin pentapeptide injections for 6 weeks. Although some immune parameters showed a tendency toward normalization no clinical improvement was observed.
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A lesion clinically and histologically mimicking trichilemmal horn was observed in the nail apparatus of a 62-year-old woman. The term 'onycholemmal horn' is proposed for this lesion.
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