Abstract:
:GM1-gangliosidosis (GM1) is one of the metabolic storage diseases, of which a differential diagnosis requires an array of biochemical assays to determine the enzyme deficiency. This approach is not only time-consuming and costly but also unavailable to most hospital laboratories. However, a presumptive diagnosis of GM1 may be made on the basis of coarse facial feature, foamy endothelial cells in the cutaneous blood vessels and ectopic Mongolian spots, if present. A more definitive diagnosis of GM1 is then made on the demonstration of deficiency of GM1 beta-galactosidase in leukocytes, plasma or cultured skin fibroblasts. Thus, a battery of enzyme tests may be averted.
journal_name
Acta Derm Venereoljournal_title
Acta dermato-venereologicaauthors
Tang TT,Esterly NB,Lubinsky MS,Oechler HW,Harb JM,Franciosi RAdoi
10.2340/0001555573412415subject
Has Abstractpub_date
1993-12-01 00:00:00pages
412-5issue
6eissn
0001-5555issn
1651-2057journal_volume
73pub_type
杂志文章abstract::Biological drugs are expensive, but can reduce symptoms and increase quality of life for patients with psoriasis. The aim of this study was to examine quality of life, disease severity and treatment satisfaction in Danish, Finnish and Swedish patients with psoriasis. Based on 12 months' data from patient surveys and c...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章,多中心研究
doi:10.2340/00015555-1485
更新日期:2013-07-06 00:00:00
abstract::The effect of anti-Parkinson therapy on the urinary excretion of 5-S-cysteinyldopa (5SCD), a catechol metabolite of dihydroxyphenylalanine (DOPA) and marker for the melanocyte, was studied by means of high performance liquid chromatography. 5SCD was normal in Parkinson patients treated with anticholinergics. DOPA admi...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1983-01-01 00:00:00
abstract::Zinc deficiency was produced in rats by feeding them a diet containing 0.5 mg zinc per kg. After 2 weeks the zinc-deficient rats showed markedly stunted growth as compared with control rats receiving an identical diet supplemented with 50 mg zinc per kg. After about 3 weeks all rats in the zinc-deficient group develop...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1980-01-01 00:00:00
abstract::Mast cells are thought to be involved in neurogenic inflammation in skin, and numerous neuropeptides are known to degranulate mast cells. We monitored histamine release in skin in situ with the microdialysis method after skin challenge with neuropeptide injections (10 microM substance P, vasoactive intestinal peptide ...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/0001555576205209
更新日期:1996-05-01 00:00:00
abstract::The burning mouth syndrome is characterized by an unpleasant sensation of burning in the oral cavity, without clinical signs. Causal factors may be psychogenic, systemic or local. The aim of the study was to determine the significance of contact allergy in the pathogenesis of burning mouth syndrome. Fifteen patients w...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/0001555576488490
更新日期:1996-11-01 00:00:00
abstract::In a patient affected with acrodermatitis continua Hallopeau, acitretin (Ro 10-1670) monotherapy resulted in a complete clearance of pustulation at a dosage of 45 mg per day. At this dosage the leukotriene B4-induced intraepidermal accumulation of polymorphonuclear leukocytes was markedly inhibited. ...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1989-01-01 00:00:00
abstract::A case of prolonged gonococcal speticemia is presented. Lack of genital symptoms, of demonstrable gonococcal antibodies in whole serum, and initially also of skin lesions contributed to a delayed diagnosis that was finally confirmed by positive blood culture. Evidence of circulating immune complexes was obtained by th...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1975-01-01 00:00:00
abstract::Patients with clinical features of dorsal macular amyloidosis but without subepidermal amyloid deposits were followed for 2-11 years. The clinical appearance was fairly stable during this period of time, with little tendency of healing. Only 2 of the patients developed typical macular amyloidosis during the follow-up....
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/0001555576302304
更新日期:1996-07-01 00:00:00
abstract::Loricrin, the major component of the cornified envelope, is normally expressed in the granular layer of epidermis during the last steps of keratinocyte differentiation. Using an antiloricrin antiserum (A8-73), an increased expression of this envelope precursor was found in some disorders of hyperorthokeratosis (ichthy...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:102340/0001555572407409
更新日期:1992-11-01 00:00:00
abstract::Cinnamic alcohol and cinnamic aldehyde are a cause of allergic contact dermatitis in man and give rise to similar rates of positive reactions in routine patch testing. However, data from animal models indicates that the aldehyde is the stronger sensitizer of the two. Circumstantial evidence has pointed to the conversi...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:102340/0001555572264265
更新日期:1992-08-01 00:00:00
abstract::The aim of this retrospective study was to determine the type and prevalence of vascular patterns in the ulcerated and non-ulcerated portions of histologically proven basal cell carcinomas (BCCs) and correlate them with other dermoscopic and clinical features, including the clinically supposed diagnosis. Three authors...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-2609
更新日期:2017-05-08 00:00:00
abstract::Previous studies on skin topically photosensitized with trimethylpsoralen and subsequently irradiated with long-wave UV light have demonstrated an increase in melanosome size and changes in the distribution patterns of melanosomes, suggesting the possibility of gene derepression or the induction of a somatic mutation ...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1977-01-01 00:00:00
abstract::Fifteen patients with nodular prurigo were treated with a trioxsalen bath (50 mg/150 l of water) and UVA given in an ordinary PUVA cabin. Good results were observed in 8 and moderate in 7 patients in the initial phase. The result was good or excellent in 13 of the 15 patients during maintenance treatment. The good res...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1979-01-01 00:00:00
abstract::Psychological stress and atopic dermatitis (AD) symptoms appear to form a vicious cycle. This study compared the degree of stress and impairment of dermatology life quality between patients with AD and healthy controls, and examined for neuropeptides and neurotrophins associated with stress in AD. Questionnaires, comp...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-0933
更新日期:2010-11-01 00:00:00
abstract::Despite advances in atopic dermatitis (AD) treatments, research into AD prevention has been slow. Systematic reviews of prevention strategies promoting exclusive and prolonged breastfeeding, or interventions that reduce ingested or airborne allergens during pregnancy and after birth have generally not shown convincing...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-3516
更新日期:2020-06-09 00:00:00
abstract::While localized variants of granuloma annulare are typically self-limited, disseminated granuloma annulare tends to be chronic and often therapy-resistant. Treatment with fumaric acid esters is effective for severe forms of psoriasis. Disseminated granuloma annulare has also been reported to respond to fumaric acid es...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-0647
更新日期:2009-01-01 00:00:00
abstract::Gel nail polish is commonly used in manicures; how-ever, research into the side-effects of gel nail polish is scarce and focusses mainly on allergic contact dermatitis. The aim of this study was to assess the frequency and characteristics of side-effects associated with use of gel nail polish. A self-questionnaire sur...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-3684
更新日期:2020-11-18 00:00:00
abstract::This hitherto unknown and dominantly inherited disorder is characterized by vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis. It has been present in the family reported for at least four generations. The lesions become visible in late c...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1981-01-01 00:00:00
abstract::The nickel content of food items consumed in Denmark was estimated on the basis of analysis by atomic absorption spectrophotometry. The highest concentrations (1-10 mg nickel/kg fresh weight) were found in cocoa, licorice, lucerne seeds, dried beans, peanuts, hazel nuts, sunflower seeds, oat meal and wheat bran. A die...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1986-01-01 00:00:00
abstract::Data regarding the efficacy and safety of methotrexate (MTX) in adults with localized scleroderma (LoS) is scarce. This study gathered data from a retrospective cohort of adult patients with LoS (n?=?107), treated with MTX (1993-2015). MTX drug survival and predictors thereof were analysed. After 1 and 2 years, 26% an...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-2411
更新日期:2016-11-02 00:00:00
abstract::Studies on hydroxyproline and hydroxylysine, the two amino acids characteristic of collagen and related glycoproteins, were undertaken on biopsies of pathologic and clinically normal human skin. No statistically significant differences between clinically normal skin of mamma, thorax, axilla, femur, hip and sacral area...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1976-01-01 00:00:00
abstract::An observational, questionnaire-based, cross-sectional study was performed to assess whether differences in coping behaviour (positive and negative strategies) between patients with either a recent diagnosis of malignant melanoma (MM) or with benign dermatological disease, were predictive of the diagnosis. Coping stra...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-1351
更新日期:2012-11-01 00:00:00
abstract::A trioxysalen bath is a safe alternative to systemic 8-methoxypsoralen in long-term psoralen plus ultraviolet A (PUVA) treatment. The kinetics of its main side-effect, the strong phototoxicity, has not been thoroughly studied. This study determined the degree and persistence of phototoxicity after a single 10 min bath...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.1080/000155501750376230
更新日期:2001-06-01 00:00:00
abstract::Erythema elevatum diutinum (EED) is a chronic disease with symmetrical persistent erythematous nodules and plaques primarily in an acral distribution. EED is often associated with infections, especially of streptococcals. An immunological reaction has been proposed as pathogenetic mechanism. We describe a patient, who...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1986-01-01 00:00:00
abstract::Repeated topical analgesia with EMLA 5% cream prior to the cleansing of venous leg ulcers was studied. Patients were randomly allocated to a series of 8 treatments with EMLA (n = 22) or to a control group (n = 21). A thick layer of the cream was applied to the ulcers for 30 min. At each of the 8 treatments, local reac...
journal_title:Acta dermato-venereologica
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.2340/000155555573231233
更新日期:1993-06-01 00:00:00
abstract::A case of incontinentia pigmenti achromians syndrome with associated abnormalities occurring in a 21-year-old woman is reported. Since birth she has had whorling macular, hypopigmented areas on the skin of the lateral, anterior, and posterior aspects of the trunk. Histopathological picture: the melanocytes in the depi...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1975-01-01 00:00:00
abstract::The area of UV erythema produced by a small beam head was found to increase with increasing doses. The aim was to investigate whether measurement of the area could be a more useful indicator of UV-induced damage than classic visual grading. Topical pretreatment with all-trans retinoic acid (tretinoin) and betamethason...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/0001555573273275
更新日期:1993-08-01 00:00:00
abstract::Oxidation of 5-S-cysteinyldopa by mushroom tyrosinase was studied by measuring 5-S-cysteinyldopa consumption with HPLC. 5-S-cysteinyldopa was found to be a substrate for tyrosinase, but our results suggests that a self-catalysed oxidation induced by enzymatically formed 5-S-cysteinyl dopaquinone also takes place. Dopa...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1980-01-01 00:00:00
abstract::This is the first case in the literature describing naevi spili, café-au-lait spots and melanocytic naevi aggregated along-side Blaschko's lines. The pattern of melanocytic lesions in our patient is different from the congenital pigmentary syndromes and the segmental distribution of melanocytic naevi, the quadrant dis...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章,评审
doi:10.1080/000155598443114
更新日期:1998-09-01 00:00:00
abstract::Gold sodium thiosulfate, inserted in the patch test standard series, gives a surprisingly high yield of positive reactions, about 10% of tested eczema patients, but with a low degree of clinical relevance. A histopathological study on patch test reactions was carried out in patients selected because of a combined cont...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/0001555574417423
更新日期:1994-11-01 00:00:00