Abstract:
:A 39-year-old man presented with multiple basaloid follicular hamartomas involving the right side of his body in a systematized pattern following Blaschko's lines. His right leg was 22.5 cm shorter than the left, and rudimentary pre-axial polydactyly was noted on the left hand and the right foot. The teeth of the right maxilla were hypoplastic. DNA analysis of blood lymphocytes and fibroblasts from lesional skin did not reveal any mutation in the Patched gene. On account of this case and of 8 similar cases found in th e literature, the spectrum of a distinct syndrome is delineated. Ipsilateral extracutaneous defects include cervical ribs, polydactyly, malformed thumb and disproportionate overgrowth or deficient growth of limb bones; dental anomalies in the form of anodontia, hypodontia or ameloblastoma; and cerebral defects such as mental retardation, unsteady gait, meningioma and optic glioma. The cutaneous lesions of this syndrome should not be called "basal cell naevus" as this will lead to continuing confusion with Gorlin syndrome. The molecular basis of the disorder remains to be elucidated.
journal_name
Acta Derm Venereoljournal_title
Acta dermato-venereologicaauthors
Happle R,Tinschert Sdoi
10.2340/00015555-0495subject
Has Abstractpub_date
2008-01-01 00:00:00pages
382-7issue
4eissn
0001-5555issn
1651-2057journal_volume
88pub_type
杂志文章abstract::Atopic dermatitis (AD) has a big impact on quality of life. The usefulness of health-related quality of life questionnaires for children with AD in general practice, and the relationship of quality of life to disease severity, as assessed by parents and by investigators, however, is not known. This study used the Infa...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-1026
更新日期:2011-03-01 00:00:00
abstract::Forty-three nickel-allergic patients (40 females and 3 males) participated in this study which was designed to elucidate the correlation between quantitative in vivo and in vitro responses. All were patch-tested with various concentrations of nickel-sulphate serial-diluted in water. The lowest concentration eliciting ...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1985-01-01 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::Using the mouse, photoallergy to the antiarrhythmic agent quinidine could be induced following systemic administration. After pretreatment with cyclophosphamide 150 mg/kg, groups of 5-10 mice were injected i.p. with quinidine chloride 100 mg/kg on 2 consecutive days, followed by exposure of shaved abdominal skin to UV...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1988-01-01 00:00:00
abstract::The effect of five corticosteroid ointments on epidermal thickness was studied using occlusive chamber application. Thinning of the epidermis and telangiectasia were noted 3 weeks after application of betamethasone-17-valerate (BV), budesonide (BD), clobetasol-17-propionate (CP), and fluocinolone acetonide (FA), but n...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1984-01-01 00:00:00
abstract::Suction blisters were raised in lesions and normal appearing skin of patients with psoriasis. The blister roof which contains the epidermis separated at the dermal-epidermal junction was stained with ATPase, OKT-6 and anti-HLA-DR monoclonal antibodies. The technique permits the counting of the Langerhans' cells per mm...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1985-01-01 00:00:00
abstract::Dyschromatosis symmetrica hereditaria (OMIM127400) is a rare autosomal dominant pigmentary genodermatosis caused by mutations in the RNA-specific adenosine deaminase (ADAR) gene. This study investigated 5 families and 3 sporadic patients with dyschromatosis symmetrica hereditaria in the Chinese Han population from Anh...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-0168
更新日期:2007-01-01 00:00:00
abstract::Dermal cells maintained in cultured human skin explants for up to 10 weeks were studied by electron microscopy. Common findings in the cell organelles were: 1) altered nuclear shapes and numerous nuclear bodies; 2) reduced rough endoplasmic reticulum; 3) a prominent Golgi complex; and 4) an increasing number of free r...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1980-01-01 00:00:00
abstract::Eighty-two cases of nodular panniculitis of the legs were examined clinically, histologically and immunohistologically. Clinically the cases could be divided into four groups: typical erythema nodosum (ENty) (35 cases), erythema nodosum migrans (ENmi) (11 cases), erythema induratum (EI) (11 cases) and the remaining 25...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1977-01-01 00:00:00
abstract::Dupilumab, the first biologic approved for treatment of atopic dermatitis, has demonstrated significant clinical effect and quality of life-enhancing capacity in clinical trials. In these, dupilumab-associated conjunctivitis where reported in a minority of patients. The present case series describe 10 patients treated...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-3121
更新日期:2019-04-01 00:00:00
abstract::Having observed altered itch and flare reactions after histamine application in patients with atopic eczema, we tried to determine these reactions in patients with urticaria and psoriasis. We investigated 16 healthy non-atopic subjects, 16 atopics in an eczema-free interval, 16 with acute atopic eczema, 16 with urtica...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.1080/000155598433458
更新日期:1998-03-01 00:00:00
abstract::Sweat from 8 'rusters' and 8 control persons was examined for its sodium concentration. Sweating was induced by iontophoresis of pilocarpine on the distal forearm. In no case did the concentrations exceed the normal upper limit for this procedure, and no difference was found between the two groups. In this way the stu...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1979-01-01 00:00:00
abstract::A 31-year-old woman developed typical clinical and laboratory signs of PCT at the end of her second pregnancy coincident with the summer season. She had elevated liver function values without history of alcoholism, hepatitis or chemical liver damage. She had taken oral contraceptive only before her first pregnancy whi...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1984-01-01 00:00:00
abstract::The forearm skin of 10 Caucasians and 10 American Negroes was inoculated with Candida albicans and the severity of the ensuing dermatitis as well as the population of Candida and other aerobes in the inoculum site have been assessed. Negroid skin proved to be less susceptible to irritation, even though it harboured a ...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1988-01-01 00:00:00
abstract::The Scandinavian Mycosis Fungoides Study Group has treated 21 patients with mycosis fungoides in plaque stages with topical, whole-body application of nitrogen mustard, 20 mg in 40 ml water per square metre. Ten patients were treated after previous attempts at intravenous tolerance induction ad modum van Scott & Kalma...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1979-01-01 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
abstract::The proactive use of topical anti-inflammatory (TAI) therapy to address subclinical inflammation is an effective, contemporary clinical strategy for the management of atopic dermatitis (AD). The interaction of a proactive TAI dose with the subclinical epidermal barrier defect in AD is yet to be determined. A randomise...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章,随机对照试验
doi:10.2340/00015555-2048
更新日期:2015-07-01 00:00:00
abstract::The pathogenesis of seborrhoeic dermatitis is controversial and remains unclear. Malassezia is considered to be a commensal fungi and is found not only in the stratum corneum but also in hair follicles. It is an important pathogenic factor in seborrhoeic dermatitis. The aim of this study was to clarify the pathogenesi...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-3519
更新日期:2020-06-15 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::Blood glutathione-peroxidase (GSH-Px) was determined in 61 healthy subjects and 506 patients with various skin disorders. Depressed levels were observed in patients with psoriasis, eczema, atopic dermatitis, vasculitis, mycosis fungoides and dermatitis herpetiformis. Low values of GSH-Px were also found in some patien...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1982-01-01 00:00:00
abstract::In this prospective study, 51 patients suffering from psoriasis and ichthyosis were treated with acitretin for 2 years. The average dose was 0.5 mg/kg/day. We have evaluated the efficacy and side effects, focusing on skeletal side effects. X-ray examinations were done before treatment and after 1 and 2 years. Forty-fi...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:102340/0001555572445448
更新日期:1992-11-01 00:00:00
abstract::The skin reactions to skin provocation tests with 11 primary irritants were compared in 21 pairs of men and women with hand eczema, matched for age, and in 21 pairs of non-eczematous subjects. With a few exceptions, no statistically significant differences were demonstrated in the intensities of the skin reactions bet...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:102340/0001555555191194
更新日期:1975-01-01 00:00:00
abstract::Prevalence and severity of seborrhoeic dermatitis were studied in 150 patients with psychiatric disorders, including schizophrenia, mood disorders, anxiety and organic mental illness. As a control group, we examined 150 patients waiting for surgery and regarded as obviously anxious. Thirty-eight psychiatric patients w...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:102340/0001555570432434
更新日期:1990-01-01 00:00:00
abstract::A 10-year follow-up of patients with juvenile plantar dermatosis and gluteo-femoral eczema disclosed a worse prognosis than previously assumed, with a course in some cases protracted into adult age. The presence of atopy was established with a clinical point score system. It was found that 52% of the patients with juv...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1988-01-01 00:00:00
abstract::Multicentric reticulohistiocytosis is a rare systemic disease of unknown aetiology characterized by erosive arthritis and cutaneous lesions consisting of multiple reddish-brown papules and nodules, mainly involving the face and distal upper extremities. It has been suggested that skin eruptions in multicentric reticul...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-0961
更新日期:2011-03-01 00:00:00
abstract::Most dermatologists are aware of the benefits of dermoscopy, and a few are familiar with laser-scanning confocal microscopy. Beyond confocal, there are fully 11 different categories of optical techniques that have been applied to clinical dermatology. This article first provides a comprehensive tabular overview of all...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-2717
更新日期:2017-01-01 00:00:00
abstract::Keratinocyte growth in vitro and DNA synthesis by epidermal cells in vivo are inhibited by therapeutic doses of cyclosporin A (CsA). This effect may be potentialized by topical treatment with ketoconazole, since this drug has been shown to inhibit CsA metabolism. Normal human skin grafts on nude mice receiving intrape...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/0001555574257259
更新日期:1994-07-01 00:00:00
abstract::Facial melanoma is difficult to diagnose and dermatoscopic features are often subtle. Dermatoscopic non-melanoma patterns may have a comparable diagnostic value. In this pilot study, facial lesions were collected retrospectively, resulting in a case set of 339 melanomas and 308 non-melanomas. Lesions were evaluated fo...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:10.2340/00015555-2759
更新日期:2017-11-15 00:00:00
abstract::The skin absorption of clioquinol and chloroquinaldol in ointment or cream base was studied in fourteen patients with widespread dermatoses. A serum concentration in the range of 0.3--1.3 microgram/ml using clioquinol and 0.05--0.1 microgram/ml with chloroquinaldol was reached by the second day and persisted throughou...
journal_title:Acta dermato-venereologica
pub_type: 杂志文章
doi:
更新日期:1978-01-01 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