[Persistent light reactivity after prochlorperazine treatment].
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Biomedical subjects
Publications and source records attributed to F Brandrup.
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A case of reactive perforating collagenosis in an adult male patient with chronic renal failure. Pruritic, umbilicate papules, showing extrusion of collagen fibers through the epidermis were detected histopathologically. Electron microscopy showed absence of basal membrane beneath the perforation and collagen fibers with preserved periodicity passing through widened intercellular spaces with islands of cytoplasmic material.
The ultrastructure of human affected and unaffected psoriatic epidermis was studied in skin biopsies from 5 patients and 3 normal controls. Transmission electron microscopic investigations revealed abnormalities in all cell layers of the affected epidermis. Common to psoriatic keratinocytes from affected epidermis was the reduction of tonofilaments. The essential ultrastructural changes were located in the stratum granulosum and stratum corneum. Thus, absence of the fusion between the keratohyalin granules and the tonofilaments was found in stratum granulosum. The keratinocytes of the stratum corneum showed a large accumulation of ribosomes and vesicles resembling lipid vesicles.
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Dermatome shaving was performed in 20 patients with chronic recalcitrant plaque psoriasis. The level of shaving was just beneath the superficial dermal vascular plexus. Healing was achieved in 2 weeks. After 1 year, six patients had shown no recurrence; 10 patients had a few guttate elements in the shaved area, while four patients had significant recurrence. Recurrences were related to residual acanthotic epidermis left after shaving. A general recommendation of surgical treatment for psoriasis would be hazardous on the basis of these results. However, shaving may constitute an alternative treatment of recalcitrant plaques of limited extent.
In 33 adult patients with atopic dermatitis of mild to severe degree the concentration of IgE in serum (S) and intercellular fluid (IF) was studied by suction blister technique. The median value of IF/S-IgE was 0.20 and not different from the ratio 0.21 found in controls. Furthermore, by subdividing the patients into groups with asthma/rhinitis or different severities of eczema no significant differences in the IF/S-IgE ratios were found compared with the controls. In the controls the IF/S-IgE ratio (0.21) was significantly different from the IF/S-IgG ratio (0.32). The median ratio of IF/S-IgE divided by IF/S-IgG was 0.63, which might indicate a reduced passage of IgE into IF compared with IgG presumably dependent on differences in the molecular size or tertiary structure of the proteins.
A case of chronic granulomatous disease (CGD) in a 32-year-old female with two episodes of opportunistic infections is described. At the age of 29 the patient was suspected to be a carrier of X-linked CGD on the basis of discoid lupus erythematosus-like skin lesions. No respiratory burst activity, as measured by phorbol myristate acetate stimulated superoxide production, was observed in isolated neutrophils of the patient. Membrane-rich fractions elicited no superoxide production in the presence of NADPH. The neutrophil content of cytochrome b-245 was within normal range. Family investigations revealed neither cellular abnormalities nor any history of skin diseases or opportunistic infections in first degree relatives. The parents of the patient were first cousins. On the basis of family history and the in-vitro assessment of neutrophil function, the patient is believed to have autosomal recessive CGD. The presented case illustrates that lupus erythematosus-like skin lesions are not restricted to female carriers of X-linked CGD, but may also be found in the autosomal recessive type of the disease.
A detailed clinical study of 76 males with steroid sulphatase deficiency confirmed the ichthyosis of this disorder to be identical with the classical descriptions of recessive X-linked ichthyosis. The appearance of regular ichthyotic scaling seems in most instances to be heralded by a general peeling of the skin. This characteristic feature was registered between age 1 and 3 weeks in 19 of 21 young boys followed due to a placental steroid sulphatase deficiency. Later on, polygonal scales giving a 'dirty' appearance were most pronounced on the distal parts of the body and extensor aspects of the extremities. The ichthyosis aggravates through childhood into adult age.
Recessive X-linked ichthyosis (RXLI) is consistently associated with steroid sulphatase deficiency, and a definite diagnosis can be made by measurement of the activity of this enzyme, e.g. in cultured skin fibroblasts and leucocytes. Demonstrating an increased electrophoretic mobility of plasma low-density lipoprotein in RXLI patients has been proposed as a simpler method for the diagnosis of this condition. Our findings in 7 RXLI patients and 7 normal controls confirmed that a discrimination between patients and controls can be obtained by routine lipoprotein electrophoresis. However, due to variation in the results of repetitive performances further studies are needed to evaluate the overall reliability of this diagnostic approach.
A 74-year-old woman with a chronic, severe, erosive lichen planus on the tongue, buccal mucosa and toes was treated successfully with dapsone. Previous therapy had failed, Partly due to side effects.
Lymphomatoid papulosis is usually considered to have a benign course, but many reports of subsequent evolution into systemic lymphoma have been reported. By measuring single-cell DNA content by flow cytometry, it may be possible to predict those cases that have the potential for the development of a malignant neoplasm. Two cases that differ from classic benign lymphomatoid papulosis had a more "malignant" clinical picture, with nodules and tumors, and the finding of aneuploidy (abnormal DNA content) from several skin lesion specimens and also from a lymph node specimen in one of the cases. Clinically evident malignant neoplasms have not yet developed in the two patients, but we suggest that the finding of aneuploidy predicts those cases that later could become malignant.
Seventy-six ichthyotic male patients with a biochemically confirmed diagnosis of steroid sulphatase deficiency are reported. Ascertainment was based on either a previous diagnosis of placental steroid sulphatase deficiency (21 probands and 15 secondary cases), or ichthyosis with steroid sulphatase deficiency (29 probands and 11 secondary cases). The ichthyotic phenotype of the first group was indistinguishable from that of the other group, and completely fitting the classic description of recessive X-linked ichthyosis. A prominent skin peeling in early infancy was found to be a characteristic feature of this syndrome. Maldescent of the testis was registered in 9 patients; and testis cancer had been diagnosed in 2 males with normally descended gonads. This high proportion of patients with gonadal abnormalities strongly indicates a relation with the steroid sulphatase deficiency. Corneal opacities, not affecting visual acuity, were seen in 14 out of 28 males by slit-lamp examination.
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