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Showing papers in "British Journal of Dermatology in 1981"


Journal ArticleDOI
TL;DR: Despite all cases with physical urticaria having been excluded, physical factors such as exercise were considered by 20% to make the urticarial attacks worse and food was mentioned as a factor worsening the weals by 30% and drinks by 18%.
Abstract: A questionnaire was used from 1972-1978 for 330 consecutive patients with recurrent urticaria of 3 months to 40 years duration. Fifty men and fifty women had only urticaria. The rest had both urticaria and angio-oedema and most of them were women between 24 and 38 years of age. Urticarial attacks were less frequent during the daytime. A personal history of rhinitis, asthma or atopic dermatitis was recorded in more than one-third. Nasal polyps, migraine and arthralgia were found in 6-7% of the patients. Severe psychiatric problems were mentioned by 16%. Abdominal problems, mainly gastritis, were described by 44%. A history of side effects from drugs was found in 32% of the patients. Food was mentioned as a factor worsening the weals by 30% and drinks by 18%. Fruits, vegetables and nuts were the most common. Despite all cases with physical urticaria having been excluded, physical factors such as exercise were considered by 20% to make the urticaria worse. Provocation tests with various food additives such as azo dyes, benzoates, butylated hydroxytoluene (BHT), butylated hydroxyanisole (BHA) sorbic acid, quinoline yellow, carotene, canthaxanthine, annatto and nitrite revealed one or more positive reactions in one-third of the patients, one-third showed negative provocation tests and, in the rest, one or several tests were questionable. Routine history of other diseases. The fibrin microclot generation test which test which indicates the presence of circulating endotoxins was positive in 24%.

340 citations


Journal ArticleDOI
TL;DR: The differences in distribution of PN in the newborn infant and that in theAdult that have been previously published suggest that most of the PN found in the adult are not congenital.
Abstract: SUMMARY Pigmented naevi (PN) were clinically diagnosed in 1083 newborn infants from a series of 531,831 consecutive livebirths examined in fifty-nine hospitals in South America. Isolated PN (IPN) were seen in 989 (0.2%) non-malformed infants, while PN associated with other congenital anomalies (APN) were observed in ninety-four (1.0%) malformed babies. Incidence rates calculated from the fifty-nine hospitals were very variable due to under-reporting, which might have been as high as 80%. The PN were classified by their location, number, size, texture, and colour. Observed locations were compared with random expected distributions based on the surface proportions of the newborn. IPN were preferentially found on the chest and abdomen, with a low concentration on the head and upper limbs. No examples were observed on soles or palms. IPN were single in 90% of cases. Size was small (1–9 mm) in 29%, medium (10–;40 mm) in 63%, and large (over 40 mm) in 8% of cases. Abnormal texture, (rugose, raised, and/or hairy) was found in 7–11% of IPN. Colour was brown in 68%, black in 21%, and of other colours in 11%. The large IPN tended to be multiple in number and abnormal in texture. Small IPN tended to be black. IPN on the chest and abdomen were more frequently small, non-black, and of normal texture; those found on the back tended to be black and abnormal in texture; black IPN were also frequent on the upper limbs; large IPN frequently affected the lower limbs. The APN did not differ from the IPN in their number, size, texture, or colour. APN were less common than expected on the back and upper limbs. An association was observed between the PN and the following four congenital anomalies: ear deformities, preauricular appendages, angiomas, and other skin anomalies. The differences in distribution of PN in the newborn infant and that in the adult that have been previously published suggest that most of the PN found in the adult are not congenital.

311 citations


Journal ArticleDOI
TL;DR: Patients with lichen sclerosus and atrophicus have an increased incidence of organ specific antibodies, and these patients and their relatives have a significantly higher incidence of associated autoimmune diseases than a control population.
Abstract: Patients with lichen sclerosus and atrophicus have an increased incidence of organ specific antibodies, and these patients and their relatives have a significantly higher incidence of associated autoimmune diseases than a control population. The incidence of other auto-immune diseases in patients with lichen sclerosus is highest when the onset is between the ages of 41 and 60 years, but is not related to the duration or site of the lesions. These findings suggest an immunological basis for the disease and indicate that the patients should be investigated and followed up to detect the existence of other auto-immune disorders.

184 citations


Journal ArticleDOI
TL;DR: A group of patients presenting with dermatological complaints but with no significant objective dermatological pathology on examination are described, with complaints related to the face were burning, intense itching and hirsutes.
Abstract: A group of patients presenting with dermatological complaints but with no significant objective dermatological pathology on examination are described. Twenty-eight patients, twelve male and sixteen female, age range 16--76 years, mean 46 years, were seen. Symptomatology was confined to three main body areas: the face--eight patients; scalp--nine patients; perineum--eight patients. The complaints related to the face were burning, intense itching and hirsutes. Scalp symptoms included excessive hair loss and intense irritation. Genital symptoms included itching, excessive redness, burning and discomfort, which in three instances prevented the patient sitting. A disturbed body image (dysmorphophobia) was common and the most frequent psychological illness present was depression. Two patients were demented and in two a diagnosis of schizophrenia was made. One patient committed suicide and two patients attempted suicide. Female patients presenting with facial symptoms have a more ominous prognosis, both with regard to the risk of suicide and the development of psychosis. It is important that dermatologists recognize this common group of patients with dermatological non-disease. Those patients who are anxiously preoccupied with their skin may be managed by superficial psychotherapy and antidepressants. Those patients who are truly deluded should be referred to a psychiatrist.

161 citations


Journal ArticleDOI
TL;DR: Initially the vibrissa cells demonstrated singular characteristics, including poor spreading ability, before eventually forming a fibroblast‐like population of cells, which is compared with cultured feather dermal papillae.
Abstract: SUMMARY Vibrissa dermal papilla cells have been successfully grown and serially cultured and their early behaviour compared with cultured feather dermal papillae. Initially the vibrissa cells demonstrated singular characteristics, including poor spreading ability, before eventually forming a fibroblast-like population of cells.

157 citations


Journal ArticleDOI
TL;DR: The results indicate that well‐developed lesions of lichen planus are characterized by an influx of helper/inducer T lymphocytes and increased numbers of Langerhans cells, supporting the contention that cellular immunity is important in the pathogenesis of this disorder.
Abstract: SUMMARY Skin biopsies from four patients with lichen planus were studied using monoclonal antibodies directed against T lymphocytes Anti-T1 and anti-T3 antibodies, which react with all peripheral T cells, stained most cells in the dermal infiltrates The majority of infiltrating cells also stained with anti-T4 and anti-T4b antibodies, which react with helper/inducer cells, whereas a minority of cells stained with anti-T8 antibody, which reacts with cytotoxic/suppres-sor cells Surface IgM was not identified on any infiltrating cells, providing evidence against B cell participation Intraepidermal and dermal cells with long cytoplasmic extensions stained with anti-T6 antibody in all cases, defining them as Langerhans cells or their precursors T6-positive cells were seen in greater number than in normal control epidermis and dermis The results indicate that well-developed lesions of lichen planus are characterized by an influx of helper/inducer T lymphocytes and increased numbers of Langerhans cells These observations support the contention that cellular immunity is important in the pathogenesis of this disorder

150 citations


Journal ArticleDOI
TL;DR: In females, antithyroid antibodies were associated with extensive hair loss: they were found in 42% of female patients with total alopecia and only 20% of males with total hair loss.
Abstract: SUMMARY The prevalence of auto-antibodies against thyroid constituents, gastric parietal cells, smooth muscle cells, mitochondria, reticulin, nuclear constituents and rheumatoid factor in 108 patients with alopecia areata was compared with that found in a previous survey of the local population. Female patients had a significantly increased prevalence of anti-thyroid antibodies which were present in 30% overall and in 44% of the youngest age group (11–17 years). Smooth muscle antibodies were more frequent in female patients but the increase was not significant. Male patients had a significantly increased prevalence of thyroid and gastric parietal cell antibodies (11.4% each). In females, antithyroid antibodies were associated with extensive hair loss: they were found in 42% of female patients with total alopecia and only 20% of males with total hair loss. A family history of alopecia areata was obtained from 24% of patients; 10% had relatives with thyroid disease and 10% had diabetic relatives. These findings confirm the association between alopecia areata and the other auto-immune diseases.

120 citations


Journal ArticleDOI
TL;DR: After a single exposure, epidermal Langerhans cells showed loss of fine dendritic processes, which may explain the impaired contact hypersensitivity observed in patients with psoriasis receiving PUVA therapy.
Abstract: SUMMARY The numbers and morphological appearance of epidermal Langerhans cells (LCs) were studied in twenty-five patients with psoriasis receiving treatment with 8-methoxypsoralen (8-MOP) and long wavelength UV irradiation (UV-A) (PUVA). After a single exposure, LCs showed loss of fine dendritic processes. Repeated treatments resulted in a reduction of the number of LCs from the mean pretreatment value of 713/mm2 to less than 60/mm2 after seven treatments. The number of LCs remained low while treatment continued for up to 4 weeks. This finding may explain the impaired contact hypersensitivity observed in patients with psoriasis receiving PUVA therapy.

117 citations


Journal ArticleDOI
TL;DR: In order to investigate the molecular basis of substrates, and the inhibitory specificity of tyrosinase, a large series of phenolic compounds have been analysed by using a High Performance Liquid Chromatographic‐Scanning Spectrophotometric system.
Abstract: In order to investigate the molecular basis of substrates, and the inhibitory specificity of tyrosinase, a large series of phenolic compounds have been analysed by using a High Performance Liquid Chromatographic-Scanning Spectrophotometric system. Depending on their chemical structure, phenolic compounds may act as substrates or as competitive inhibitors of tyrosinase. The ability to act as substrates requires the presence in the molecule of electron donor groups, while competitive inhibition on the contrary requires the presence of powerful electron acceptor groups. Certain phenolic compounds used as therapeutic agents or as food preservatives are chemically capable of acting as alternative substrates or competitive inhibitors of tyrosinase in vitro; their effect on melanocytes in vivo therefore merits investigation.

117 citations


Journal ArticleDOI
TL;DR: For the first time a method for culturing human hair follicle cells is described, using the bovine eye lens capsule as the substrate for the cultures.
Abstract: For the first time a method for culturing human hair follicle cells is described. The bovine eye lens capsule, a basement membrane-like structure, is used as the substrate for the cultures. In a culture medium supplemented with hydrocortisone and insulin about 70% of the original follicles will form growing colonies of diploid keratinocytes.

89 citations


Journal ArticleDOI
TL;DR: A significant loss of demonstrable surface β2 microglobulin from the surface of malignant cells compared to benign, and a partial loss in the premalignant cases.
Abstract: SUMMARY Biopsies from twenty-three patients with malignant skin lesions and from twenty-two patients with premalignant or benign skin lesions were stained for β2 microglobulin by use of the immunoperoxidase technique. The results showed a significant loss of demonstrable surface β2 microglobulin from the surface of malignant cells compared to benign, and a partial loss in the premalignant cases. This difference could prove to be a useful tool in the histological diagnosis of malignant skin lesions, and in assessing whether or not the lesion has been completely excised.

Journal ArticleDOI
TL;DR: Microscopic studies have been performed on skin biopsies from five patients with occupational argyria and found no significant differences in the content of the cells found in the blood of these patients.
Abstract: SUMMARY Microscopic studies have been performed on skin biopsies from five patients with occupational argyria. Small brown-black granules were present in the dermis on light microscopy and were intensely refractile with dark-field illumination. Electron microscopy showed that the granules were electron-dense, round or oval in shape and varied in size from 30 nm to 100 nm. They were most numerous in relation to the basal lamina of the eccrine sweat glands, but were also present in relation to the basal lamina of the epidermis and dermal elastic fibres. X-ray microanalysis confirmed that many of the granules contained silver and sulphur. However, selenium, mercury, titanium and iron were also identified and it is probable that these elements were deposited in the skin also as a result of occupational exposure.

Journal ArticleDOI
TL;DR: Retinoid alone proved to be the least effective, showing, however, a good to excellent result in 65% of patients, and another advantage achieved by RePUVA was a highly significant reduction of total UVA doses to about one third as compared to PUVA therapy.
Abstract: Eighty ambulatory adult patients with severe psoriasis were included in our 14-week study to compare the clinical effects of treatment with an aromatic retinoid (Tigason), combination treatment with aromatic retinoid plus PUVA (RePUVA), and PUVA alone. RePUVA was given by two different modifications: either 4 weeks of pretreatment with retinoid followed by 6 weeks of PUVA treatment, or 10 weeks of retinoid treatment with concomitant PUVA treatment during the last 6 weeks. The latter modification of RePUVA proved to be significantly more effective than the other treatments (P less than 0.01) as regards complete remission. With respect to the number of patients with good or excellent improvement (75-100% improvement) there was no marked difference between RePUVA and PUVA treatments. Retinoid alone proved to be the least effective, showing, however, a good to excellent result in 65% of patients. Besides the increase in efficacy, another advantage achieved by RePUVA was a highly significant (P less than 0.001) reduction of total UVA doses to about one third as compared to PUVA therapy.

Journal ArticleDOI
TL;DR: Seven new eases showing unilateral systematized, linear or zosteriform distribution of lesions resembling Darier's disease, but without other findings suggestive of Darier’s disease are presented.
Abstract: SUMMARY In this report seven new eases showing unilateral systematized, linear or zosteriform distribution of lesions resembling Darier's disease, but without other findings suggestive of Darier's disease, are presented. It is suggested that the eruption represents an epidermal naevus for which the name acantholytic dyskeratotic epidermal naevus is proposed.

Journal ArticleDOI
TL;DR: In this article, five patients with Behcet's disease were satisfactorily treated with colchicine and reported improved oral aphthosis, erythema nodosum-like lesions and genital erosions.
Abstract: Five patients with Behcet's disease were satisfactorily treated with colchicine. Oral aphthosis, erythema nodosum-like lesions and genital erosions improved greatly within a month as did laboratory findings. We believe colchicine to be the first choice in the management of the cutaneous and ocular lesions of Behcet's disease.

Journal ArticleDOI
TL;DR: Dapsone yielded excellent therapeutic results in certain forms of lupus erythematosus (LE), whereas discoid lesions and the maculo‐papular rash of the systemic and disseminated chronic forms of discoid LE remained uninfluenced by the drug.
Abstract: Dapsone yielded excellent therapeutic results in certain forms of lupus erythematosus (LE), whereas discoid lesions and the maculo-papular rash of the systemic and disseminated chronic forms of discoid LE remained uninfluenced by the drug. On the basis of these observations, we suggest the following indications for dapsone treatment in LE: (1) Vasculitic urticaria. (2) Oral ulceration. (3) Non-scarring form of chronic LE. (4) Chloroquine intolerance.

Journal ArticleDOI
TL;DR: Age‐related changes in the human dermal elastic fibres were studied by scanning (SEM) and transmission (TEM) electron microscopy and showed an increase in the complexity of shape and arrangement of the fibres including flattening and branching, an increased in the roughness of the surface, and a decrease in interfibrillar areas.
Abstract: Age-related changes in human dermal elastic fibres were studied by scanning (SEM) and transmission (TEM) electron microscopy. The SEM findings showed an increase in the complexity of shape and arrangement of the fibres including flattening and branching, an increase in the roughness of the surface, and a decrease in interfibrillar areas. The TEM findings showed a decrease in micro-fibrils and amorphous material, and an increase in electron dense inclusions followed by the appearance of vesicular structure.

Journal ArticleDOI
TL;DR: The staining pattern in cutaneous amyloidosis was morphologically distinctive and readily distinguishable from staining of thickened vascular basement membranes in porphyria, and may become the procedure of choice in the differential diagnosis of amyloidsosis.
Abstract: The presence of amyloid P component (AP) in dermal deposits of cutaneous amyloidosis was demonstrated by a direct immunofluorescence technique using an antibody to serum amyloid P component (SAP). AP was also shown, for the first time, to be a constituent of normal human skin. It was present at the periphery of dermal elastic tissue fibres, in basement membranes of dermal blood vessels and surrounding eccrine sweat glands but was absent from the dermo-epidermal basement membrane. The staining pattern in cutaneous amyloidosis was morphologically distinctive and readily distinguishable from staining of thickened vascular basement membranes in porphyria. Immunofluorescence with anti-SAP is simple and specific and may become the procedure of choice in the differential diagnosis of amyloidosis.

Journal ArticleDOI
TL;DR: A review of 124 cases of bullous pemphigoid was made and the clinical findings correlated with the results of indirect immunofluorescence, but antibody titres in these patients did not correlate with the extent of the disease or with the presence of mucous membrane lesions.
Abstract: A review of 124 cases of bullous pemphigoid was made and the clinical findings correlated with the results of indirect immunofluorescence. A circulating IgG basement membrane zone (BMZ) antibody was detected in the serum of eighty-nine (72%) of the patients. Antibody titres in these patients did not correlate with the extent of the disease or with the presence of mucous membrane lesions. Indeed, oral lesions were more common in the thirty-five sero-negative patients with 17% of such patients being affected compared to only 10% of those with positive indirect immunofluorescence findings. This difference, however, was not statistically significant. Concurrent malignant disease (i.e. malignancy occurring within 6 months of pemphigoid being diagnosed) occurred in eight of the seronegative group (23%) and in only four of the eighty-nine seropositive cases (4%); this difference was significant. The association of bullous pemphigoid and malignant disease still remains controversial, but those who believe that the relationship is coincidental must now explain why concurrent malignant disease is more common in patients who have bullous pemphigoid and negative indirect immunofluorescence findings.

Journal ArticleDOI
TL;DR: The clinical and histopathological features of nineteen cases of Zoon's balanitis and a comparable lesion of the vulva have been reviewed and recognition of these features should allow simple differentation from premalignant, infective and other inflammatory penile lesions which may be more responsive to treatment.
Abstract: The clinical and histopathological features of nineteen cases of Zoon's balanitis and a comparable lesion of the vulva have been reviewed. The condition is particularly distinguished by characteristic histological changes affecting the epidermis and the dermal vasculature. The presence of a plasma cell infiltrate is relatively non-specific. Recognition of these features should allow simple differentiation from premalignant, infective and other inflammatory penile lesions which may be more responsive to treatment.

Journal ArticleDOI
TL;DR: The skin and oral mucosa were studied in an unselected series of carriers of X‐linked chronic granulomatous disease, a hereditary condition in which phagocytic cells display a pronounced functional defect.
Abstract: The skin and oral mucosa were studied in an unselected series of carriers of x-linked chronic granulomatous disease, a hereditary condition in which phagocytic cells display a pronounced functional defect. Three carriers had discoid lupus erythematosus (DLE)-like skin lesions which histopathologically were consistent with DLE of the hypertrophic and profundus type. Four patients had experienced photosensitivity in childhood. Seven patients had recurrent aphthous-like stomatitis which should be distinguished from the recurrent aphthous stomatitis seen in otherwise healthy individuals. The remarkably high incidence of DLE-like symptoms in heterozygous carriers might be related to the presence of mixed populations of defective and normal phagocytes. The variable expression of skin symptoms may be related to uneven distribution of abnormal and normal phagocytes. Female patients with these clinical symptoms, especially the combination of DLE-like skin lesions and aphthous-like stomatitis, should be suspected of being carriers of chronic granulomatous disease and studies of phagocyte function in vitro should be performed, since the diagnosis of the carrier state is of utmost importance for genetic counselling before pregnancy.

Journal ArticleDOI
TL;DR: The analysis of the intrauterine growth curves showed that PN infants have a low prematurity rate, and a high mean birth weight within the postmature group, lacking the normal post‐term weight loss pattern shown by the control newborn group, suggesting that congenital PN appear in the latest stages of intrauterines development.
Abstract: Genetic and environmental risks factors were investigated in a sample of 989 non-malformed newborn infants with skin pigmented naevi (PN), and in 989 non-malformed non-PN, control babies. The samples were obtained from a population of 531,831 livebirths, from fifty-nine South-American maternity hospitals. No significant differences were observed between the PN and control groups for the following risk factors: sex, twinning, parental consanguinity, socioeconomic level, parental ages, birth order, fetal presentation, type of delivery, postnatal mortality, and first trimester of pregnancy histories for maternal chronic illnesses or immunizations, radiation exposure, drug intake, and vaginal bleeding. PN infants were associated with high frequencies of Black racial ancestry, positive prenatal history for maternal acute illnesses, and high mean values for length of gestation and birth weight. The observed association of PN with maternal acute illnesses could be a spurious one, due to partial dependence upon Black racial ancestry, and to maternal memory bias. The analysis of the intrauterine growth curves showed that PN infants have a low prematurity rate, and a high mean by the control newborn group. This observation suggest that congenital PN appear in the latest stages of intrauterine development. No evidence was obtained to allow us to consider congenital PN as true developmental anomalies.

Journal ArticleDOI
TL;DR: Tetracycline, declomycin and erythromycin, in concentrations lower than the minimal inhibitory dosages, were shown to inhibit lipase production by Propionibacterium acnes in vitro.
Abstract: Summary Tetracycline, declomycin and erythromycin, in concentrations lower than the minimal inhibitory dosages, were shown to inhibit lipase production by Propionibacterium acnes in vitro.

Journal ArticleDOI
TL;DR: Two patients with urticaria evoked at the site of contact of skin with water have been studied and protection of the skin from contact with water by prior application of petrolatum ointment prevented wealing, but removal of the stratum corneum enhanced wealing.
Abstract: SUMMARY Two patients with urticaria evoked at the site of contact of skin with water have been studied. Protection of the skin from contact with water by prior application of petrolatum ointment prevented wealing, but removal of the stratum corneum enhanced wealing. Organic solvents did not themselves evoke wealing, but they enhanced the reaction to subsequent challenge by water. That the release of acetylcholine is an essential step in the pharmacogenesis of wealing in aquagenic urticaria is indicated by the suppressive effect of locally-applied scopolamine on water–evoked wealing. Aquagenic urticaria is also associated with elevated blood histamine levels and degranulation of mast cells in the water–challenged skin. The relationship of acetylcholine and histamine to each other and to contact of water with the skin remains uncertain.

Journal ArticleDOI
TL;DR: Patients with disabling disorders of keratinization treated with Tigason (etretinate, Ro 10‐9359), an oral aromatic retinoid, and the clinical responses and the effects on the skin were monitored suggest that the drug acts at a late stage of epidermal differentiation.
Abstract: Twenty patients with disabling disorders of keratinization were treated with Tigason (etretinate, Ro 10-9359), an oral aromatic retinoid, and the clinical responses and the effects on the skin were monitored. Most patients showed a considerable clinical improvement within 4 weeks. Side effects, such as cheilitis, were common but mostly transient or minor. In the skin there was a decrease in glucose-6-phosphate dehydrogenase activity within the granular cell layer of the epidermis, and an increase in mean epidermal thickness and mean corneocyte area. However, there was little apparent effect on epidermal proliferation or on histological and ultrastructural appearances. These findings suggest that the drug acts at a late stage of epidermal differentiation.

Journal ArticleDOI
TL;DR: Chromosome analysis of HSC‐i, frozen and thawed H SC‐1, the transplanted tumour in a nude mouse and HSC-1b all showed hypotetraploidy with the modal number 80, which reconfirms that HSC•1 derives from the original skin tumour.
Abstract: A cell line of a squamous cell carcinoma of human skin (HSC-I) has been established in vitro, and successfully maintained proliferative in continuous tissue culture for about 2 1/2 years since March 1978. Another cell line (HSC-Ib) has been established in vitro from the recurrence of the same cancer. It has been maintained for about 8 months since December 1979. 3T3 feeders were used in the initial cultures in both cases. The cells grow in a monolayer in vitro and are epithelioid with anaplastic features. Chromosome analysis of HSC-I, frozen and thawed HSC-I, the transplanted tumour in a nude mouse and HSC-Ib all showed hypotetraploidy with the modal number 80, which reconfirms that HSC-I derives from the original skin tumour. The cell line is available to other investigators.

Journal ArticleDOI
TL;DR: Most anaphylactic disorders, allergic rhinitis, asthma, allergic urticaria and angio-oedema, and anaphlyactic gastro-enteritis, are mediated by IgE reaginic antibodies, and its properties are well known.
Abstract: Most anaphylactic disorders, allergic rhinitis, asthma, allergic urticaria and angio-oedema, and anaphylactic gastro-enteritis, are mediated by IgE reaginic antibodies. An antibody mediating anaphylactic sensitivity binds to specific receptors on mast cell and basophil membranes by the Fc portion of the molecule, leaving the Fab portion free to react with antigen. IgE is the classic example of an anaphylactic antibody i its properties are well known.

Journal ArticleDOI
TL;DR: The pedigree supports X‐linked recessive inheritance and close linkage with the Xg2 locus was excluded and normal chromosomal stability was found in three patients and immunological studies precluded an early universal defect in cell‐mediated immunity.
Abstract: SUMMARY This kindred includes six males with dyskeratosis congenita. It is the largest British pedigree so far reported and brings the total number of reported cases to fifty-nine. Our pedigree supports X-linked recessive inheritance and close linkage with the Xg2 locus was excluded. Three previously unreported complications are noted; Hodgkin's disease, adenocarcinoma of the pancreas and deafness. Normal chromosomal stability was found in three patients and immunological studies precluded an early universal defect in cell-mediated immunity.

Journal ArticleDOI
Ronald Marks1
TL;DR: The numbers of cells in the epidermis and their height decreased with age, whereas the surface area of individual corneocytes tended to increase with increasing age, which may be responsible for some of the alterations noted clinically in the skin of elderly individuals.
Abstract: SUMMARY Measurements were made of the thickness of the epidermis, the dimensions of keratinocytes and of the surface areas of individual corneocytes in normal human skin from three non-light exposed sites. The numbers of cells in the epidermis and their height decreased with age, whereas the surface area of individual corneocytes tended to increase with increasing age. The changes demonstrated may be responsible for some of the alterations noted clinically in the skin of elderly individuals. Measurement of tissue ageing is of importance both to the interpretation of the effects of diverse external stimuli and to the study of the effect of dietary and chemical manipulations on the ageing process. Many of the tests devised measure ageing in individual tissues or organ systems and ‘test batteries’ have been employed to obtain an overall assessment of ageing in the organism as a whole (Comfort, 1979). Tests that measure a complex function such as the ‘hand grip strength test’ or determine a biochemical parameter summate the effects of disease, cumulated environmental traumata and true biological ageing and cannot in themselves be used to measure the ageing process. Histometric techniques have the advantage that inspection of the tissue allows identification of the effects of disease and exclusion if the effects of ageing alone are to be considered. They have the disadvantage of any invasive technique and in addition are time consuming. Skin is an ideal organ for the investigation of age dependent changes because of its accessibility. In addition, because of its admixture of tissue types and the different functions that it subserves it is possible to obtain a broad picture of the changes due to ageing. This study attempts to define age related change in overall epidermal dimensions and in parameters of keratinocyte and corneocyte size.

Journal ArticleDOI
TL;DR: Light microscopic examination of 1 μm thick epoxy resin sections of human skin preserved in fixative normally used for electron microscopy allows division of the microvasculature of the reticular and superficial dermis into several distinct segments.
Abstract: SUMMARY Light microscopic examination of 1 μm thick epoxy resin sections of human skin preserved in fixative normally used for electron microscopy allows division of the microvasculature of the reticular and superficial dermis into several distinct segments. More precise delineation of these components can be achieved by correlative study using electron microscopy. Measurement of vessel diameter in tissue sections is of limited value in vessel classification because there is a wide overlap between different vascular segments and the results are influenced by the type of fixative used for processing the tissue. Detailed observation of the morphological characteristics of the endothelium and its investment, including the elastic and basal laminae, is considered preferable for identifying individual vascular segments. Differences in body region do not appear to have any obvious influence on vessel structure although there are regional differences in vessel density and arrangement. In comparison with earlier studies on animal tissues or human skin, no structures resembling arteriovenous shunts or precapillary sphincters were observed in the present investigation. However, closed fenestrae and other structures thought to be transendothelial channels were often found at the tips of capillary loops adjacent to the epidermis.