Depigmentation: its significance in patients with melanoma.
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Biomedical subjects
Publications and source records attributed to L Barnes.
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We describe a 19-year-old girl who has clinical features of microphthalmia with linear skin defects (MLS) syndrome caused by a microdeletion of Xp22.3. In addition to the classical ocular abnormalities and linear skin defects she has other features not previously described. She was previously reported in this journal in 1990 as poikiloderma congenitale, but her true diagnosis of an Xp22.3 microdeletion was clarified when fluorescent in situ hybridization (FISH) analysis indicated that one of her X chromosomes had a microdeletion including the KAL gene. We describe this patient with an Xp22.3 microdeletion to heighten awareness among dermatologists of this syndrome and to underscore the difficulties in diagnosing MLS syndrome.
The efficacy and safety of long-term concurrent twice-daily treatment of scalp and body psoriasis with calcipotriol scalp solution (50 mcg/ml) and calcipotriol cream (50 mcg/g) were evaluated in a prospective, multi-centre, open-label, non-controlled evaluation over 52 weeks in 202 patients. Safety and efficacy as measured by total sign score (scalp psoriasis), modified PASI (body psoriasis) and patient self-assessment were assessed at week 2, 6 and 10 and thereafter every six weeks. By week 28, mean total sign score for scalp psoriasis had reduced from 5.9 to 2.5 (p<0.001). No further reduction was seen. By week 34, mean PASI for body psoriasis had reduced from 6.8 to 2.6 (p<0.001). No further reduction was seen. At week 52, the percentage of patients assessing their psoriasis as moderate or severe had decreased from 72 to 21% for scalp psoriasis and from 62 to 19% for body psoriasis. Facial irritation was the most frequent adverse event (91/276 events) with the highest incidence occurring at week 2 and few new reports at subsequent visits. There were no significant changes in mean serum calcium, parathormone or urinary calcium/creatinine ratio. Combined treatment with calcipotriol scalp solution and cream was effective and safe for long-term treatment of scalp and body psoriasis.
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PURPOSE: To characterize the MR features of skull base chordomas with regard to signal intensity, size, position, extension, and Gd-DTPA enhancement. PATIENTS AND METHODS: The MR imaging studies of 28 patients with surgically proven chordomas of the skull base were retrospectively reviewed. Twenty-two of these patients received intravenous administration of Gd-DTPA. RESULTS: On short TR/short TE images, chordomas generally had low to intermediate signal. On long TR/long TE images, chordomas generally had very high signal that was heterogeneous in 79%. After Gd-DTPA administration, all chordomas demonstrated some degree of contrast enhancement. In most cases, enhancement was demonstrated throughout most of each tumor in a heterogeneous pattern. Chordomas were associated with MR findings of displacement and encasement of vessels, and frequent extension into adjacent structures such as the cavernous sinus, sella, nasopharynx, and hypoglossal canal. CONCLUSION: The MR characterization of the position and extent of these neoplasms played an important role in determining the optimal surgical approaches for gross total tumor resection.
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Psoriasis is a common condition in the western world, but is uncommon in populations with a low prevalence of atherosclerosis such as Japanese, Blacks and indigenous North Americans. Psoriasis appeared to be more prevalent amongst patients attending our lipid clinic compared to the general population. Lipoprotein (a) (Lp(a)) is elevated in conditions such as coronary heart disease and protein loosing nephropathies. Psoriasis is a condition of increased epidermal protein turnover and loss and thus it might be postulated that increased levels of Lp(a) may be found in patients with psoriasis. The aim of this study was to explore an association between psoriasis and Lp(a) and lipid profiles. No association was found between Lp(a) concentrations and psoriasis. Although six of 36 patients were found to have a cholesterol greater than 7.5 mmol L-1, the mean cholesterol for this group was not elevated.
Previous Irish studies have shown a high prevalence of nonmelanoma skin cancer (NMSC). However, unlike elsewhere where the generally quoted ratio of basal cell carcinoma (BCC):squamous cell carcinoma (SCC) is at least 4:1 the ratio in Ireland was found to be 1:1. We examined this ratio in patients attending the dermatology outpatients and determined risk factors for the development of NMSC within an Irish population where 75% of the population have been shown to have skin type 1 or 11. The study consisted of a retrospective 10 year review of biopsy proven cases of NMSC and a prospective case control study of NMSC which allowed examination of risk factors and comparison to a control group. The BCC:SCC ratio in the retrospective study was 3:1. In the prospective study the ratio of BCC:SCC was 4.4:1. Skin type 1 and numerous freckles were identified as risk factors in the NMSC group.
A retrospective clinical follow-up study of 50 cutaneous melanomas diagnosed at a Pigmented Lesion Clinic over a five year period is presented. Compared to previous Irish studies the mean Breslow depth of the tumours was considerably less, thus indicating that patients presenting to such clinics have more superficial melanomas and ultimately a better prognosis.
A prospective study was carried out between January 1988 and January 1990 in St. James's hospital, Dublin, which is the chief referral centre for the HIV population in Ireland. Patients were routinely referred from the genitourinary medicine department to the dermatology clinic for a full dermatological assessment which was carried out by one physician (LB). The patients were not preselected on the basis of a skin complaint. The study aimed to detect the prevalence of skin disease amongst this HIV group of patients and to compare them with an age and sex matched group of normal controls. There were 92 patients in each group, with 62 intravenous drug users (IVDU), 21 homosexuals, eight haemophiliacs and one heterosexual in the HIV positive group. A wide spectrum of skin disorders were seen, with a significantly higher prevalence of seborrhoeic dermatitis, xerosis, Kaposi's sarcoma, oral candidiasis, folliculitis, molluscum contagiosum, onychomycosis, gingivitis, perianal herpes simplex and genital/perianal warts in the HIV positive group compared to controls.