Penile and scrotal swelling in a child.
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Biomedical subjects
Publications and source records attributed to S Cliff.
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BACKGROUND: Hyperhidrosis (primary or secondary) is excessive sweating beyond that required to return body temperature to normal. It can be localized or generalized, commonly affecting the axillae, palms, soles or face, and can have a substantial negative effect on a patient's quality of life. IMPACT OF DISEASE: Objective evaluation comprising quantitative assessment (gravimetric and Minor's iodine starch test) and subjective evaluation (Dermatology Quality of Life Index and Hyperhidrosis Impact Questionnaire) allow accurate assessment of the impact of hyperhidrosis on patients. BOTULINUM TOXIN TYPE A: Botulinum toxin type A acts by inhibiting the release of acetylcholine at the presynaptic membrane of cholinergic neurones. It has proved useful in treating a number of diseases relating to muscular dystonia and is now proving beneficial in treating hyperhidrosis. Clinical trials investigating botulinum toxin type A use in axillary and palmar hyperhidrosis show significant benefits with few side-effects reported, with a favourable impact also being seen on patient quality of life. Botulinum toxin type A injections are generally well-tolerated with beneficial results lasting from 4 to 16 months. CONCLUSIONS: Botulinum toxin type A injections are an effective and well-tolerated treatment for hyperhidrosis. This paper proposes a positioning of this treatment along with current established treatments, and highlights the role of botulinum toxin type A as a valuable therapy for the treatment of hyperhidrosis.
BACKGROUND: Angiogenesis is a prerequisite for growth of invasive tumours. We hypothesized that angiogenesis would be present in invasive basal cell carcinoma (BCC) but not in a noninvasive tumour such as actinic keratosis (AK). OBJECTIVES: To investigate both types of tumour for evidence of angiogenesis. METHODS: Patients with BCC or AK underwent intravital videocapillaroscopy. Three regions were examined: the tumour, perilesional skin and a control site. Microvessel width, area fraction and length density were determined from capillaroscopy images. Biopsies were stained for CD34 and a microvessel count was performed. RESULTS: Capillaroscopy demonstrated a grossly disorganized tumour microcirculation in BCC. Compared with control skin, microvessel width was increased 2.4-fold, area fraction was increased 4.9-fold and length density was increased 5.9-fold. In AK, microvessel width was increased 1.7-fold, area fraction 2.5-fold and length density 3.4-fold. Vessel width and area fraction were significantly greater in BCC than AK. Biopsies showed significant increases in microvessel length density for both BCC and AK compared with control skin, with BCC significantly greater than AK. CONCLUSIONS: Angiogenesis was demonstrated in BCC in humans in vivo, and to a lesser extent in AK.
Skin cancer is increasingly common, and the skills involved in its diagnosis should be promoted in UK medical schools. However, there has been no scientific evaluation of the teaching methods employed by dermatology departments. The aim of this study was to evaluate, using traditional audiovisual methods, the impact of an illustrated booklet on skin cancer, coupled with a lecture, on undergraduates' diagnostic skills. The ability of 27 final-year medical students to recognize a variety of skin lesions, using projected images from clinical slides, was assessed. They were tested without warning on two occasions. Immediately after the first test, students were given an illustrated booklet on skin tumours and pigmented lesions which was supplemented with a lecture based on the booklet. Two weeks later, a second test was employed using a series of slides deemed to be of equal diagnostic difficulty. Our results showed a significant increase in the median number of correct diagnoses between the first and second tests (P < 0.001). However, there remained wide variation at the second test in the percentage of correct answers (30 to 80%) amongst students. Our study highlights the need to develop effective methods for improving the diagnostic skills of undergraduates in dermatology, and the importance of evaluating teaching methods. The methods of evaluation, such as ours, can be simple and inexpensive.
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Hereditary multiple glomus tumours constitute an autosomal dominant skin disease which is known to demonstrate cutaneous mosaicism typified by type 1 and 2 segmental arrangements. We report a patient with type 2 segmental multiple glomangiomyomas who was disturbed by the pain of her lesions. A symptomatic lesion was successfully treated with the pulsed dye laser and to date there has been no recurrence of the pain. Possible explanations for the clinical response are discussed.
Seventeen general practitioners (GPs) were evaluated to assess their ability to recognize malignant, borderline and benign skin lesions before and after a novel, cheap and quick skin cancer educational programme. They were tested without prior warning on two occasions using two sets of 30 clinical slides. Between each test the GPs were given a lecture based on an illustrated booklet of similar lesions. The results showed an improvement in the GPs' diagnostic skills (P < 0.05), but nevertheless a wide variation in diagnostic ability between GPs remained. Our study highlights a simple, effective and inexpensive method for teaching GPs the diagnostic clinical features of skin cancer. Further work is needed to improve their diagnostic accuracy in the long term.
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Angiogenesis is a recognized event in psoriasis. Previous ultrastructural studies have demonstrated lymphatic capillaries extending high into the dermal papillae. Using the novel method of fluorescence microlymphography which permits visualization of upper dermal initial lymphatics in vivo we tested the hypothesis that lymphangiogenesis exists within plaque psoriasis. Six patients underwent fluorescence microlymphography with fluorescein isothiocyanate-dextran administered intracutaneously within a psoriatic plaque on the leg. Stereological analysis permitted quantification of the lymphatic network opacified both within (lesional) and without (perilesional) the plaque. Results showed a greater spread of tracer from the depot into perilesional skin than into the plaque (P < 0.006). The mean length of lymphatics per unit area at increasing distance from the centre of the depot was also increased for the perilesional skin, 10.5 +/- 1.9/cm2 (mean +/- SEM), compared with lesional skin, 3.06 +/- 0.8/cm (P < 0.001). The cumulative lymphatic length was also greater in perilesional, 22 +/- 7.3 cm2, compared with lesional skin, 3.6 +/- 0.3 cm (P < 0.006). Fluorescence microlymphography has proved to be an effective in vivo technique for the assessment of the dermal microlymphatics in psoriasis. Stereology provided quantitative analysis of the lymphatic network visualized. Overall, there is a greater network of lymphatics in perilesional compared with lesional skin in patients with plaque psoriasis. This finding is at odds with the accepted view that the lymphatic dermal vessels are increased within the psoriatic plaque.
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BACKGROUND: Pyoderma gangrenosum (PG) is an uncommon necrotising, non-infective ulceration of the skin. The management of PG is aimed at limiting tissue destruction, promoting the healing of the wound, and providing an acceptable cosmetic result. However, skin grafting is normally avoided because of the potential risk of pathergy-the localization of skin damaged by trauma. REPORT: We describe the use of split skin grafts in the management of ulcerative pyoderma gangrenosum in 4 patients. RESULTS: Our cases demonstrate that split skin grafts are a useful treatment modality in patients with ulcerative PG, producing a good cosmetic result. One case illustrates the importance of ensuring the disease is quiescent prior to grafting, to avoid pathergy. The other cases emphasise the need for prolonged immunosuppressive therapy to minimise the chance of reactivation of the disease process. CONCLUSION: Our preliminary experience of 4 cases of ulcerative PG indicates that split skin grafts have a role to play in its management. The ultimate cosmetic result is considered to be superior to allowing the wound to heal by secondary intention. To limit the risk of pathergy developing, our experience suggests a role for prolonged courses of immunosuppressive therapy. The most effective dose and duration of immunosuppressive therapy in patients with PG treated with split skin grafts remains to be determined. A controlled study would be of benefit to compare it with other current treatment options.
Port wine stains (PWS) are congenital vascular naevi. Their presence is the cause of significant psychological morbidity due to their cosmetic appearance. The flashlamp-pumped dye laser (FPDL) is considered to be the treatment of choice for PWS. However, there is a recognized morbidity related to the use of the FPDL. The PhotoDerm VL is a broad spectrum, non-coherent, intense pulsed light source which has been shown to be an effective tool in the treatment of a number of vascular lesions including PWS. Our strategy was to assess the effectiveness of the PhotoDerm VL in the treatment of mature PWS in three fair skinned subjects. Three patients with mature PWS were recruited. They were treated at 6-weekly intervals with the PhotoDerm VL machine using predetermined parameters and assessed at each visit prior to treatment. In all three patients there was at least a 50% improvement in the clinical appearance of the lesions as assessed by both operator and patient. No complications were reported. Further treatments in two patients, however, failed to produce any further clinical improvement. We conclude that the PhotoDerm VL appears to be a promising treatment for PWS with no post-operative complications. However, a comparative study with the FPDL needs to be undertaken to determine the most effective therapy option for patients with this disfiguring cutaneous lesion.
Unilateral naevoid telangiectatic syndrome (UNTS) was first described in 1899 and is characterized by the dermatomal distribution of telangiectasia. It is broadly divided into congenital and acquired forms based upon the time of presentation. To date no effective treatment has been reported for this condition. In view of the vascular component of this eruption, it was felt that the pulsed dye laser (PDL), 585 nm, may have a role to play in the selective photothermolysis of these lesions. The treatment of five patients with acquired UNTS using the PDL is described. Based upon the findings it is concluded that the PDL is an effective treatment for UNTS; however, the clinical response is short lived with a 100% recurrence noted in all cases. Possible explanations for this condition are also briefly discussed.
Lupus pernio of the nose is the most characteristic cutaneous lesion of sarcoidosis. It is cosmetically disfiguring and can be the cause of significant morbidity. In particular, the affected skin is often red or purple due to increased vasculature. It is particularly resistant to both surgical and medical therapy. We describe a patient with lupus pernio affecting her nose, which showed a dramatic improvement following treatment with the pulse dye laser (PDL). A biopsy taken after treatment showed the continued presence of sarcoidal granulomas. We therefore feel that treatment with the PDL is an effective tool in improving the cosmetic appearance of lupus pernio, but does not influence the underlying disease process.
The role of angiogenesis in tumour growth and metastasis is well established. However, investigations of tumour microcirculation to date have used either biopsy material from human tumours, or animal models in vivo. We have studied the tumour microcirculation in vivo in human skin cancers using video-microscopy to examine 12 basal cell carcinomas (BCCs) on the head and neck of 11 patients, and compared the vessels with those seen in the peri-lesional skin, and in normal control skin on the opposite side of the body. The vessels seen within the BCCs were markedly abnormal qualitatively, forming bizarre, disorganized patterns. Quantitative analysis revealed that the mean diameter (+/-standard deviation) of the largest vessel was significantly greater within the BCC (0.086+/-0.029 mm) than that in the control skin (0.034+/-0.012 mm) (P<0.001). The area fraction, a measure of the area of tissue occupied by vessels, was increased highly significantly within the BCCs (0.158+/-0.038) compared with both peri-lesional skin (0.029+/-0.012) and control skin (0.027+/-0.010) (P<0.001). Length density, the length of blood vessel per unit area of tissue, was also highly significantly greater within the lesion (210.22+/-66.05 cm(-1)) compared to peri-lesional (27.10+/-15.67 cm(-1)) and control skin (28.27+/-15.81 cm(-1)) (P<0.001). This is the first study to have demonstrated that BCCs possess a distinct tumour microcirculation which can be observed directly, and assessed quantitatively. Prospective studies of tumour progression, possibly after intervention with angiogenesis inhibitors, are possible.
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BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disease of unknown etiology that causes arrhythmias, heart failure, and sudden death. Diagnosis can be difficult, and this hampers investigation of its molecular basis. Forms of ARVC in which gene penetrance and disease expression are greater should facilitate genetic study. We undertook a clinical and genetic investigation of Naxos disease, originally described by Protonotarios in 1986. This disease constitutes the triad of ARVC, diffuse nonepidermolytic palmoplantar keratoderma, and woolly hair. METHODS AND RESULTS: We evaluated the population of Naxos, Greece, to identify probands, which was followed by family screening. Twenty-one affected persons from 9 families of 150 persons were identified. Linkage analysis was performed with microsatellite markers. The disease locus mapped to 17q21. A peak 2-point LOD score of 3.62 at theta=0.0 was found with a marker within intron 4 of the keratin 9 gene, a member of the type I (acidic) keratin family. A preserved homozygous disease haplotype was identified. Haplotype analysis delimited the disease interval. CONCLUSIONS: Hair and skin abnormalities were found to be reliable markers of subsequent heart disease. This suggests the presence of a single mutant gene with novel cardiac, skin, and hair function or two or more tightly linked disease genes. Recessive inheritance of Naxos disease and a founder effect were demonstrated. Identification of a fully informative genetic marker linked to the disease and uncommon in the background population may be of use as a test to identify disease gene carriers.
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