Non-bullous ichthyosiform erythroderma with annular digital constrictions.
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Biomedical subjects
Publications and source records attributed to R Marks.
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It is our belief that any abnormality in keratinization is secondary to an increased rate of epidermal cell production and decreased transit time and research into the underlying nature of psoriasis should concentrate on the issue of whether the stimulus to increased epidermopoietic activity is dermally or epidermally derived.
A technique is described which measures the penetration of substances through the stratum corneum (SC) and the distribution of elements in this structure employing scanning electron microscopy and energy dispersive analysis. Preliminary observations show that the normal SC shows a concentration gradient of potassium, high in the surface layers and lowest deeper down whereas the reverse is true for phosphorus. It has been shown that sulphur rapidly tranverses the SC and seems to penetrate through all parts of the horny layer whereas lead and zinc do not easily enter this structure.
The fatty acid composition of the skin and plasma lipids is described in a patient severely affected by Refsum's disease whose plasma phytanic acid concentration was very high (3.1 mg/ml). In the epidermal lipids, especially in the phospholipid fraction, phytanic acid tended to replace linoleic acid and to some extent arachidonic acid. In some respects the changes in the skin in Refsum's disease resemble those of essential fatty acid deficiency.
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Four patients are described who developed granulomatous reactions in the red portions of their tattoos. Histopathological and immunofluorescence studies showed features of lichen planus. Mercury was identified in only one patient's lesion, and hypersensitivity to mercury was shown by patch testing in one other patient. Tattooing may provide a localised antigenic challenge resulting in spontaneously occurring lichen planus.
Succinate dehydrogenase (SDH), glucose-6-phosphate dehydrogenase (G6PDH) and lactate dehydrogenase (LDH) activities have been studied using quantitative enzyme histochemical techniques in the epidermis of five patients with solar keratoses and Bowen's disease. 'Non sun-exposed' buttock skin was compared with the skin from the actual lesion and adjacent, clinically normal paralesional skin. SDH activity was significantly increased in the basal layer and decreased in the granular layer in the epidermis of both lesion and paralesional skin, although the total epidermal activities were unchanged when compared to 'non-exposed' buttock skin. G6PDH activity was increased in the granular layer of paralesional epidermis and of lesions. No change in LDH activity was detected. Inclusion of phenazine methosulphate in the reaction mixtures resulted in a three-fold increase in formazan deposition without altering the localization. It is concluded that the quantitative changes and alteration in localization of SDH and G6PDH reported in solar keratoses are accompanied by similar changes in adjacent, clinically normal 'sun-exposed' skin and differ from normal 'non-exposed' skin. It is suggested that these changes may precede the development of the solar keratoses and that these findings may indicate a significant metabolic alteration in the events that lead to neoplasia.
The epidermal metabolic activity of a patient with a marked generalized ichthyosis associated with heredopathia atactica polyneuritiformis has been investigated. Both the in vivo labeling index and the in vitro rates of incorporation of radioactively labeled thymidine, proline, histidine and acetate were increased relative to normal indicating a high rate of epidermopoiesis. Thin-layer chromatographic analysis of 14C-acetate containing lipid extracts revealed qualitative changes compared with normal. In particular, altered proportions of radioactivities were incorporated into the triglyceride and phospholipid moieties. However, as abnormal patterns of lipogenesis are also seen in autosomal dominant ichthyosis, these changes are probably a reflection of disordered keratinization.
The epidermis of pyogenic granulomata is presented with an unusual problem of distorted dermoepidermal relationships. In this study we have attempted to delineate the problem and have studied the way in which the epidermis copes with its difficulties. Sixteen pyogenic granulomata with (where possible) paralesional skin (and for comparison ten Campbell de Morgan spots) were studied by cell kinetic techniques. High labelling indices were found in the epidermis and in the endothelium in the pyogenic granulomata (contrasting strikingly with the findings in the Campbell de Morgan spots). A variable morphological response was found ranging from hyperplasia with parakeratosis to epidermal thinning and atrophy. Possible mechanisms for the epidermal changes are discussed and it is suggested that the data produced from this and similar studies may aid the understanding of dermo-epidermal interaction in a variety of common skin disorders including psoriasis.
The effect of various fractions of serum on active epidermal cell migration in vitro has been studied by incubating small explants of normal human skin in the presence of the fractions and determining the extent of migration after 48 h incubation. Serum was fractionated by combining ammonium sulphate precipitation and molecular sieve chromatography using Sephadex G-200. The components of serum responsible for most, if not all, of the migration promotion activity were localized in the G-200 third peak (albumin fraction) of the supernatant after precipitation of the serum with ammonium sulphate at 50% saturation.
The in vitro incorporation of 14C acetate by the epidermis has been studied in patients with autosomal dominant ichthyosis and in patients with a dry, itchy, slightly scaly skin associated with a disorder of the small bowel. Analysis of 14C acetate containing lipid moieties by thin layer chromatography has indicated that there are both quantitative and qualitative differences in the uptake of 14C acetate between autosomal dominant ichthyosis and normal. In particular an increased incorporation into the triglyceride and phosphatidyl choline fractions was noted. No such differences were apparent in those patients with disorders of the small bowel. In addition the in vitro incorporation of radioactively labelled thymidine, proline and histidine has been studied in these patients. In both groups of patients the rate of incorporation of tritiated thymidine and histidine into epidermal macromolecules was found not to differ significantly from normal. On the other hand the rate of incorporation of tritiated proline was increased in both groups of patients.
Changes in the epidermis following application of three corticosteroids, betamethasone 17-valerate, hydrocortisone 17-butyrate, and hydrocortisone have been studied histometrically in human volunteers. The reduction in epidermal thickness observed correlated significantly with a reduction in size of the viable epidermal cells. There was no significant reduction in the number of cells constituting the viable epidermis. These findings indicate that thinning of the epidermis is a function of cell size rather than cell number. The epidermal changes developed quickly and were rapidly reversible. It is suggested that measurement of cell size may be an early and sensitive index of atrophogenicity induced by topical corticosteroids. 0.1% Hydrocortisone 17-butyrate and 0.1% betamethasone 17-valerate showed equivalent potency in causing epidermal thinning and reduction in cell size. Reduction in cell size paralleled increasing concentrations of betamethasone 17-valerate, indicating a positive dose-effect relationship.
The effect of dexamethasone on the regeneration of new epithelium over the base of an excised suction blister has been studied over a period of 9 days. It was found that dexamethasone did not significantly affect the migration of epidermis over the blister base but it did significantly reduce the number of cells in DNA synthesis and post wounding acanthosis of wound edge and regenerating epithelium.
A syndrome, consisting of a congenital abnormality of the scalp, ears and nipples inherited as an autosomal dominant trait, is reported. Ten affected members of a family over five generations are recorded and the four living affected members are described in detail. A review of congenital localized aplasias of skin suggests that this may be a new syndrome.
A simple biochemical technique has been used to study the rate of incorporation of precursor materials in the involved and uninvolved skin of psoriatic subjects. Psoriatic plaques were found to incorporate tritaited thymidine twice as rapidly as skin from control subjects. The uninvolved skin of psoriatics showed a 48% increase in thymidine incorporation. Histidine and proline incorporations were also increased in psoriatic plaques but not in the uninvolved skin of psoriatics.
Transepidermal elimination is a phenomenon which occurs spontaneously in certain skin disorders in which effete connective tissue or foreign materials is expelled via the epidermis to the exterior. In order to learn how this phenomenon is effected and what factors control it, we have attempted to produce an animal model for the process. When charcoal particles are deposited subepidermally in guinea-pig flank skin transepidermal elimination occurs within 4 days and we believe that this mimics the disorder seen in man. The hair follicles became hyperplastic and were intimately involved in the reaction, some of the particles actually being expelled via the follicular lumina. The reaction has some similarities to the wound healing response.
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