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  • 1
    Keywords: EXPRESSION ; CELL ; evaluation ; Germany ; MICROSCOPY ; DISEASE ; DISTINCT ; PROTEIN ; COMPONENTS ; DIFFERENTIATION ; MARKER ; IMPACT ; SKIN ; ALPHA ; MEMBRANE ; MUTATION ; REPAIR ; COMPONENT ; ABERRATIONS ; EXTRACELLULAR-MATRIX ; MUTATIONS ; BETA ; ADHESION ; CELL-ADHESION ; HUMAN KERATINOCYTES ; INTEGRIN ; epidermis ; ultrastructure ; MATRIX ; assembly ; basement membrane ; BASEMENT-MEMBRANE ; extracellular matrix ; ARRAY ; DEFECTS ; INTEGRINS ; analysis ; methods ; JAPANESE ; DEFECT ; immunoelectron microscopy ; EXTRACELLULAR-MATRIX PROTEIN-1 ; lipoid proteinosis ; microvasculature ; aberration ; DERMO-EPIDERMAL JUNCTION ; ECM1 ; HEMIDESMOSOMES ; LAMININ ISOFORMS ; LICHEN-SCLEROSUS ; skin and oral mucosa ; VII COLLAGEN
    Abstract: Background: Excessive basement membrane (BM) deposition in skin and mucosa is characteristic for lipoid proteinosis (LP; hyalinosis cutis et mucosae), an inherited disease caused by extracellular matrix protein 1 (ECM1) mutations. According to ultrastructure there are striking differences between junctional. and microvascular BM. Objective: Distinct analysis of the junctional zone in epidermis and oral mucosa, contrasting concentric BM arrays in the microvasculature; evaluation of impact on epithelial. histogenesis and differentiation, and specifically on adhesion structures to BM (hemidesmosomes). Methods: LP-epithelia were analyzed for alterations in differentiation, BM composition and texture, and hemidesmosomal components by indirect immunofluorescence (IIF), electron microscopy (EM), and immunoelectron microscopy (ImEM). Results: Most striking was the irregular deposition of collagen IV and VII, BM-laminin, and laminin-5 at the junctional. zone, accompanied by lamellate or punctuated structures below BM (IIF), whereas integrin alpha 6 beta 4 and bullous pemphigoid antigen-1 and -2 (BPAG-1/-2) were regularly aligned. Also integrins alpha 2 beta 1 and alpha 3 beta 1 remained restricted to the epidermal basal layer, while the tissue-specific differentiation markers keratin K1/10 (mucosa, additionally K4/13) appeared delayed indicating mild hyperplasia, further confirmed by focal K6/16 expression. Ultrastructure (EM) disclosed abundance of extended basal cell protrusions and junctional aberrations like exfoliating excessive BM material. Hemidesmosomes were complete, but ImEM indicated weakened interactions between their components (BRAG-1, -2, and HD1). Confirming IIF, collagen IV and VII, and laminin-5 appeared extensively scattered, the latter two probably remaining associated. Conclusions: Subtle defects in anchorage assembly, spanning the entire BM zone, apparently compromise epithelial-matrix adhesion, which may provoke (mechanical stress-induced) erroneous BM repair. (c) 2006 Japanese Society for Investigative Dermatology. Published by Elsevier Ireland Ltd. All rights reserved
    Type of Publication: Journal article published
    PubMed ID: 17175139
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  • 2
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    German Medical Science GMS Publishing House; Düsseldorf
    In:  45. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 31. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 27. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR); 20170906-20170909; Stuttgart; DOCSpA.11 /20170904/
    Publication Date: 2017-09-04
    Keywords: ddc: 610
    Language: English
    Type: conferenceObject
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  • 3
    Keywords: EXPRESSION ; BLOOD ; Germany ; PROTEIN ; DIFFERENTIATION ; MARKER ; EXTRACELLULAR-MATRIX ; MUTATIONS ; HUMAN KERATINOCYTES ; MORPHOLOGY ; INTEGRIN ; INVOLVEMENT ; TUMOR ANGIOGENESIS ; ultrastructure ; MATRIX ; VESICLES ; extracellular matrix ; laminin ; HISTOLOGY ; function ; LOSSES ; JAPANESE ; POLARITY ; immunoelectron microscopy ; HUMAN-SKIN ; microvascular density ; basement membrane components ; EXTRACELLULAR-MATRIX PROTEIN-1 ; GENE ECM1 ; lipoid proteinosis ; microvasculature ; PERLECAN
    Abstract: Background: In lipoid proteinosis (LP) vascular anomalies represent severe functional defects caused by excessive deposition of basement membrane (BM)-like matrix, particularly around small subepithelial blood vessels. Objective: Correlation of microvascular anomalies in morphology and ultrastructure with extracellular matrix composition and cell interactions for elucidating vascular involvement in LP-pathophysiology. Methods: Biopsies from non-related LP-patients were analyzed by indirect immunofluorescence (IIF), electron microscopy (EM), and immune-EM (ImEM). Results: In LP-skin and mucosa the thickened vessel walls stained strongly for the BM-components type IV collagen, laminin, perlecan, and nidogen (IIF). Integrin (alpha 6 beta 4 was regularly collocated with endothelial surface markers such as PECAM (CD31). Ultrastructure (EM) revealed highly ordered matrix deposits around microvessels, with frequently collapsed lumina, functionally compensated by increased vascular density (histology, IIF). Pericytes were trapped between these concentric BM-layers at varying distances towards the periphery (EM), contrasting their regularly close endothelial apposition. Periodic type IV collagen patterns (ImEM) corroborated the multiple BM-leaflet structure and the lack of a common 'fused' endothelial-pericyte BM, seen normally. Presumptive secretory vesicles, abundant in both cell types, implied an equal contribution to BM-synthesis, but also indicated partial loss of endothelial polarity. Conclusions: In LP thickened vessel walls, composed of multiple BM, profoundly alter microvascular properties, also by interference with endothelial-pericyte interactions. The increased microvascular density reflects compensatory restoration for disabled function. Most remarkable was the exaggerated secretory activity (also at luminal surfaces) underlining the regulatory key role of extracellular matrix protein 1 (ECM1; mutated in LP) in export or turnover of all major BM-components. (c) 2006 Japanese Society for Investigative Dermatology. Published by Elsevier Ireland Ltd. All rights reserved
    Type of Publication: Journal article published
    PubMed ID: 16497486
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  • 4
    Keywords: SKIN ; PHOTOSENSITIVITY ; PHOTOPROVOCATION
    Abstract: This study aimed to determine whether a broad-spectrum liposomal sunscreen with a very high sun protection factor (Daylong actinica) can prevent damage induced by ultraviolet (UV) irradiation in patients with cutaneous lupus erythematosus (CLE) and healthy controls (HCs) under standardised conditions. In 20 patients with CLE and 10 HCs, defined areas of sunscreen-untreated and sunscreen-treated skin on the upper back were irradiated with combined UVA/UVB light. Disease-specific skin lesions were induced by UVA/UVB light in the untreated areas of nine patients with CLE; no specific eruptions or any sun damage was observed in the sunscreen-treated areas in any of the CLE patients, nor in the HCs. Histological analysis of skin biopsy specimens confirmed the clinical results. In conclusion, the use of a high-protection, broad-spectrum sunscreen can prevent UV-induced damage in patients with CLE and HCs.
    Type of Publication: Journal article published
    PubMed ID: 23171460
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  • 5
  • 6
    Keywords: THERAPY ; MODEL ; SYSTEM ; SYSTEMS ; NEW-YORK ; THERAPIES ; congenital ; USA ; ichthyosis
    Type of Publication: Meeting abstract published
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  • 7
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    German Medical Science GMS Publishing House; Düsseldorf
    In:  26. Fortbildungswoche für praktische Dermatologie und Venerologie; 20180724-20180728; München; DOCP65 /20180716/
    Publication Date: 2018-07-17
    Keywords: ddc: 610
    Language: German
    Type: conferenceObject
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  • 8
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Proliferierende Trichilemmalzyste Plattenepithelkarzinom ; Diagnostik Therapie ; Key words Proliferating trichilemmal cyst ; Squamous cell carcinoma ; Diagnosis ; Therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A large ulcerated tumor covered the right hemisphere of the scalp of a 77 year old woman. By histological, immunohistological, and electronmicroscopical examination, a proliferating trichilemmal cyst with focal areas of a poorly differentiated squamous cell carcinoma was identified. Six months after exstirpation of the entire tumor and dissection of supraomohyoidal lymph nodes, metastases in nuchal and supraclavicular lymph nodes and soft tissue of the neck became apparent. The present case underlines the necessity of a preoperative diagnostic evaluation and of radical surgical treatment for proliferating trichilemmal cysts.
    Notes: Zusammenfassung Wir berichten über einen die rechte Hemisphäre einnehmenden, ulzerierenden Tumor am Kapillitium einer 77jährigen Patientin. Histologisch, immunhistologisch und elektronenmikroskopisch zeigte sich eine proliferierende Trichilemmalzyste mit Anteilen eines wenig differenzierten Plattenepithelkarzinoms. 6 Monate nach Exstirpation des Tumors in toto und supraomohyoidaler Lymphknotenausräumung kam es zur Metastasierung in die supraklaviculären und nuchalen Lymphknoten sowie in die Weichteile des Halses. Der vorliegende Fall verdeutlicht die Notwendigkeit einer gründlichen präoperativen Diagnostik und eines radikalen chirurgischen Vorgehens bei proliferierenden Trichilemmalzysten.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Der Hautarzt 50 (1999), S. 203-207 
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Myiasis ; Dermatobia hominis ; Cordylobia anthropophaga ; Furunkel ; Fernreisen ; Key words Myiasis ; Dermatobia hominis ; Cordylobia anthropophaga ; Abscess ; International travel
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Cutaneous myiasis is a temporary infestation of the skin with fly larvae. Travelling to subtropical areas accounts for a higher risk and increasing incidence in Europeans. In Middle- and South America myiasis is mainly caused by the botfly (Dermatobia hominis). Blood-suckling arthropods, usually mosquitoes, transmit the larvae of the botfly via phoresis, a unique mechanism of egg deposition. In Africa cutaneous myiasis is mostly due to the tumbu fly (Cordylobia anthropophaga). Infection with the tumbu fly larvae occurs after direct contact with the eggs that are often deposited in clothes and towels. Clinically an abscess-like lesion develops. Creeping sensations of movement under the skin are occasionally described. Following hatching, spontaneous healing can normally be expected, although extraction of the larvae is recommended to prevent abscess formation and superinfection.
    Notes: Zusammenfassung Die kutane Myiasis ist eine temporäre Infestation der Haut mit Fliegenlarven, die aufgrund des zunehmenden Fernreisetourismus in subtropische Gebiete auch vermehrt in Europa angetroffen wird. In Mittel- und Südamerika wird die kutane Myiasis meistens durch die Larven der Bot- oder Dasselfliege (Dermatobia hominis) verursacht. Moskitos oder andere blutsaugende Insekten übertragen die Larven der Dasselfliege durch Phoresis, einem einzigartigen Mechanismus der Eiablage. Im afrikanischen Raum lösen die Larven der Tumbufliege (Cordylobia anthropophaga) häufig die kutane Myiasis aus. In Kleidung oder Handtüchern abgelegte Eier der Tumbufliege führen dabei nach direktem Kontakt zur Infestation. Klinisch imponiert eine Furunkel-ähnliche Läsion, vereinzelt wird über spürbare Bewegungen in der Haut berichtet. Nach dem „Schlüpfen” der Larven erfolgt in der Regel eine spontane Heilung. Die Extraktion der Larven wird jedoch empfohlen, um stärker entzündliche Reaktionen und Sekundärinfektionen zu verhindern.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Melanom ; Gesicht ; Schnittrandkontrollierte Chirurgie ; Lokalrezidiv ; Keywords Melanoma ; Face ; Micrographic surgery ; Local recurrence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Background and objective. Malignant melanomas on sun-exposed skin are often poorly circumscribed and thus recur frequently. The aim of our clinical trial was to compare conventional to a modified micrographic surgery of primary melanoma. Patients/Methods. 28 patients with in-situ (n=7) and invasive (n=21) melanoma were treated with conventional surgical excision with wide margins; eight of these patients developed a local recurrence. In comparison, 20 patients with primary in-situ (n=7) and invasive (n=13) melanoma as well as four patients with recurrence after conventional surgery underwent modified micrographic surgery with delayed closure of the wound. Results. Paraffin specimens of the margins revealed in half of the patients remnants of melanoma although excision was extended to non-lesional skin. Within a mean follow-up period of 21.3 months, none of our patients treated by modified surgery developed a recurrence. Conclusions. Local recurrencies of melanoma on sun exposed skin may be avoided by means of a modified micrographic surgery using permanent histologic sections.
    Notes: Zusammenfassung Hintergrund und Fragestellung. Flächige Melanome sind in aktinisch geschädigter Haut zu den Seiten hin schlecht abgrenzbar und rezidivieren daher häufig. Anhand einer vergleichenden Studie stellen wir die Vorteile einer lückenlos schnittrandkontrollierten Chirurgie bei diesen Melanomen vor. Patienten/Methodik. 28 Patienten mit In-situ- (n=7) und invasiven Melanomen (n=21) wurden mit einem Sicherheitsabstand bis zu 3 cm operiert. Im Folgenden traten bei 8 Patienten Lokalrezidive auf. Im Vergleich zu diesem Kollektiv wandten wir bei 20 Patienten mit vergleichbaren In-situ- (n=7) und invasiven (n=13) Melanomen sowie 4 Patienten mit einem Melanomrezidiv ein lückenlos schnittrandkontrolliertes, 2-zeitiges operatives Vorgehen an. Ergebnisse. Die modifizierte Schnittrandaufarbeitung am Paraffinschnitt zeigte in 50% der Patienten trotz Exzision in klinisch gesunder Haut noch Melanomanteile. Bisher entwickelte keiner der Patienten innerhalb eines mittleren Nachbeobachtungszeitraums von 21,3 Monaten ein Lokalrezidiv. Schlussfolgerung. Die lückenlos schnittrandkontrollierte Chirurgie scheint die hohe Rezidivrate von Melanomen im Gesichtsbereich zu senken.
    Type of Medium: Electronic Resource
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