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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 244 (1972), S. 264-269 
    ISSN: 1432-069X
    Keywords: Melanosis circumscripta praeblastomatosa ; Ultrastructure of melanocytes ; Pigmentation ; Lipid degeneration of melanocytes ; Proliferation of the basal lamina
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 248 (1974), S. 361-378 
    ISSN: 1432-069X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die epidermale Hyperkeratose der X-chromosomal-recessiven Ichthyosis Wells-Kerr (XRI) wird ohne qualitative Störungen und ohne morphologisch faßbare Defekte der Verhornung ausgebildet, wie elektronenoptische Untersuchungen an Biopsien von 6 gesicherten Fällen von XRI gezeigt haben. Keratohyalin wird vermehrt synthetisiert; Transitzellen sind sehr viel häufiger als in gesunder Haut nachweisbar. Keratinosomen kommen dagegen in verringerter Menge vor; ihre Feinstruktur ist normal. Die Auflösung der desmosomalen Disken im Horn erfolgt verpätet (meist erst nach der 25. Lage) und beginnt fokal, was zur Abhebung paketartiger Hornzellgruppen und zur klinisch grobfeldrigen Schuppung führt. In den 70–90 Lagen sehr dünner orthokeratotischer Hornzellen ist ein normales Keratinmuster nachweisbar. — In der Diskussion und in einer Tabelle werden die wesentlichsten ultrastrukturellen Unterschiede zwischen X-chromosomalrecessiver Ichthyosis, autosomal-dominanter Ichthyosis vulgaris und autosomalrecessiver Ichthyosis congenita herausgestellt. Die XRI ist auch ultrastrukturell als eigenständiges Krankheitsbild aufzufassen.
    Notes: Summary Electron microscopic investigations of 6 cases with X-linked recessive ichthyosis Wells-Kerr (XRI) have shown, that the epidermal hyperkeratosis develops without qualitative disturbances or structural defects of keratinization. The increased synthesis of an ultrastructurally normal keratohyalin is the most impressive difference to autosomal dominant ichthyosis vulgaris (ADI). Keratinosomes are found in decreased numbers in the uppermost layers of the stratum malpighi; they are of normal ultrastructure and size. Transit cells may be regularly found and hence occur in larger numbers than in healthy skin. This observation is in accordance with the slightly increased transit time of the keratinocytes as shown by Frost. The horny layer consists of 70–90 very thin orthokeratotic cells with a normal keratin pattern. A delayed dissolution of the desmosomal disks, beginning focally at different sites after the 25tb layer of horn cells, leads to a distinct type of desquamation. The main differences between XRI, ADI, and ichthyosis congenita (lamellar ichthyosis) are discussed. XRI represents a distinct nosological entity not only from genetic and clinical, but also from ultrastructural points of view.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 281 (1989), S. 165-172 
    ISSN: 1432-069X
    Keywords: Netherton's syndrome ; Retinoid therapy ; Etretin ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A young female patient, expressing the symptom triad of Netherton's syndrome, i.e., ichthyosis linearis circumflexa Comèl, trichorrhexis invaginata and other hair shaft defects, and atopic diathesis, has been treated successfully with the new retinoid preparation Etretin. Our electron microscopical study especially focused on the ultrastructural effect on the characteristic, active part of the skin lesions, which is only found within a narrow borderline just preceding the lesion's margin. In untreated skin, this part is characterized by dermal inflammation, immigrating inflammatory cells, and specific keratinization disturbances: synthesis of keratinization proteins is suppressed, serum exudates invade the epidermis, either filling the intercellular spaces of the upper spinous and the granular layer as finely granular, amorphous material, or they are partly phagocytosed and lie within intracellular, round-oval inclusions. The portions of the lesions lying towards the center are unspecific and represent recovery stages, ultrastructurally resembling stages of normal wound repair. Oral therapy with Etretin did not heal the basic defect, but drastically reduced exoserosis and the deposition of intra- and extracellular material. Keratinization seemed to normalize. The condition of the hair was also improved.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 240 (1971), S. 61-78 
    ISSN: 1432-069X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Das „Stade éphélide“ der melanotischen Präcancerose Dubreuilh ist anhand eines einschlägigen Falles klinisch durch eine progressive pigmentierte Makel charakterisiert. Histologisch findet man nur eine Hyperpigmentierung der basalen und suprabasalen Zellschichten der Epidermis. Ultrastrukturell ist diese Form der melanotischen Präcancerose aber durch eine Proliferation pathologischer Melanocyten in der Basalzellschicht charakterisiert. Die Syntheseaktivität der Melanocyten ist erhöht und die reifen Melaningranula haben von der Norm abweichende Formen, wie sie auch bei fortgeschritteneren Stadien der melanotischen Präcancerose beobachtet werden können.
    Notes: Summary The so-called „Stade éphélide“ of precancerous melanosis is clinically characterized by a progressively growing hyperpigmented freckle as shown by an own case of this type. Histopathologically there is nothing more than a simple hyperpigmentation of the basal and suprabasal cell layer of the epidermis. By electron microscopy this early form of precancerous melanosis reveals a proliferation of pathological melanocytes in the basal layer. There is an increase in synthetic activity of these melanocytes and their melanin granules are different in shape and size from those occurring in normal skin. They resemble melanin granules from later stages of precancerous melanoses (Lentigo melanosis).
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 240 (1971), S. 123-137 
    ISSN: 1432-069X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Biochemische und elektronenoptische Untersuchungen wurden an einem 20 jährigen Patienten mit einem kompletten oculocutanen Albinismus durchgeführt. Durch eine 4 wöchige UV-Stimulation kombiniert mit α-Methyl-Dopa undl-Dopa konnte eine deutliche Zunahme normal strukturierter Melanocyten dargestellt werden, während jegliche Anzeichen einer Pigmentinduktion fehlen. Vor und nach Stimulierung sind die Prämelanosomen und andere Zellorganellen der Melanocyten normal strukturiert. Eine eindrückliche Zunahme der Golgi-Zonen, des ribosomenbesetzten endoplasmatischen Reticulums, der Mitochondrien und Prämelanosomen konnte durch die Stimulierung im gleichen Ausmaß erreicht werden, wie dies bei normaler Haut der Fall ist. Der Schutz der Albinohaut durch die Lichtschwiele ist im Vergleich zum Schutzeffekt von Lichtschwiele mit Pigmentierung bei normaler Haut sehr schwach. Die Struktur der Melanocyten und deren Organellen sind beim oculocutanen Albinismus normal und können auch normal stimuliert werden. Der Defekt liegt auf der Stufe der Tyrosinase oder deren Verwendung. Elektronenoptische Untersuchungen erlauben den oculocutanen Albinismus vom partiellen Albinismus abzugrenzen (Piebaldismus und Chediak-Higashi-Syndrom).
    Notes: Summary Biochemical and electron microscopical studies were done in a 20 year old male patient with complete albinism. The number of normally structured melanocytes was elevated by a four week stimulation by UV irradiation combined with α-methyl-Dopa and l-Dopa, but there was no sign of induction of pigmentation. Before and after stimulation the melanocytes contain normally structured premelanosomes and other organelles. There is an impressive increase in Golgi areas, of rough surfaced endoplasmic reticulum, mitochondria and premelanosomes in the melanocytes after stimulation. The protection of albino skin by the “Lichtschwiele” is weak in comparison to its combined effect together with pigmentation in normal caucasian skin. In oculocutaneous albinism the melanocytes are structurally normal and can be stimulated in the normal way. There must be a defect of tyrosinase or its transfer to the premelanosomes, perhaps by an inhibitor. By electron microscopy the oculocutaneous albinism can easily be distinguished from partial albinism in piebaldism and in the Chediak-Higashi-syndrome.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 246 (1973), S. 77-91 
    ISSN: 1432-069X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Der von Curth u. Macklin (1954) publizierte Fall einer autosomal-dominanten hystrixartigen Ichthyose wurde im Rahmen einer klinischen und familiengenetischen Nachuntersuchung histopathologisch und elektronenmikroskopisch analysiert. Die Ultrastrukturbefunde zeigen, daß es sich um eine eigenständige nosologische Entität handelt. Sie unterscheidet sich durch zwei kennzeichnende Störungen der Keratinisierung von den übrigen erblichen Ichthyosen: a) Ausbildung konzentrischer geschlossener Tonofibrillenschalen, b) Vorliegen zweikerniger Zellen in allen suprabasalen Lagen der Epidermis als Folge von Zellteilungsstörungen bei erhöhter Teilungsrate der Keratinocyten.
    Notes: Summary This paper reports histopathological and ultrastructural findings of a case of autosomal dominant hystrix-like ichthyosis. It has been published for the first time in 1954 by Curth and Macklin and was reinvestigated in combined clinical, morphological, genetical, and linkage studies. This type represents a distinct nosological entity differing from all other types of inherited ichthyoses. It is characterized by two major disturbances of keratinization: a) a segregation of the cytoplasmic components of keratinocytes resulting in concentric unbroken shells of tonofilaments surrounding the nucleus and a small rim of cytoplasm with ribosomes, endoplasmic reticulum, and mitochondria; b) a disturbance of cell division after the nuclear mitotic separation, resulting in binuclear cells in about one third of all keratinocytes. In the horny layer, keratinization is less extensively disturbed than in bullous congenital erythroderma.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 246 (1973), S. 317-327 
    ISSN: 1432-069X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Fibrilläre Körper, aus der Histopathologie als „hyaline“ oder „kolloide“ Körperchen bekannt, wurden vereinzelt bei verschiedensten Dermatosen, sowie in großer Menge bei einem Fall von melanotischer Präcancerose mit ausgeprägter Pigmentinkontinenz in der Dermis und in verschiedenen Höhen der Epidermis nachgewiesen. Die Befunde werden im Sinne einer epidermalen Genese der fibrillären Körper gedeutet. Der strukturelle Zusammenhang eines intraepidermalen fibrillären Körpers mit intakten Desmosomen zeigt, daß diese Körper aus Tonofibrillen bestehen und somit von Keratinocyten abstammen.
    Notes: Summary Fibrillar bodies, known from histopathology as “hyalin” or “colloid” bodies, are shown to occur in large numbers within the cutis and, less frequently, within the epidermis in a case of precancerous melanosis with pronounced pigmentary incontinence. The present findings support the epidermal origin of these bodies. Structural continuity of an intraepidermal fibrillar body with normal desmosomes indicates that these bodies are composed of tonofibrils and thus are derived from epidermal keratinocytes.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 279 (1987), S. S38 
    ISSN: 1432-069X
    Keywords: Porokeratosis Mibelli ; Disseminated, bilateral, hyperkeratotic variant of PM ; Autophagocytic cells ; Dyskeratotic cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Clinical, histopathological, and ultrastructural features of a new clinical variant of porokeratosis Mibelli (PM) are presented. We report a solitary case of a patient, male aged 62, who developed disseminated verrucous nodules on the buttocks and the lower extremities 3 years before diagnosis. Histopathologically, all specific signs of the keratinization disorder of PM were demonstrable; in addition, however, multiple cornoid lamellae were found at the margin as well as in the center of the lesions, which only in part showed relationships to the epidermal appendages. In the papillary dermis, numerous ectatic capillaries were conspicuous. Using electron microscopy the same specific abnormalities of the keratinization process as known from classical cases of PM could be demonstrated: autophagocytic cells that revealed perinuclear edematization and vacuolization, accumulation of autophagic vacuoles and heterolysosomes, and dyskeratotic corps ronds-like cells that become transformed to fibrillar or Civatte bodies. Problems of the classification, differential diagnosis, and pathomorphogenesis are discussed.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 243 (1972), S. 88-100 
    ISSN: 1432-069X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die epidermale Keratinisierung wurde an 3 Fällen von Ichthyosis congenita und an 1 Fall eines Sjögren-Larsson-Syndroms elektronenoptisch untersucht. Der gesteigerten Proliferationsrate entsprechend können Mitosen auch au Ultradünnschnitten häufiger beobachtet werden. Die Feinstruktur solcher Teilungsstadien weist auf einen normalen Ablauf der Zellteilung hin. Die elektronenoptisch faßbaren Störungen der Keratininisierung sind im Bereich der Malpighischicht quantitativer Natur und als Folge der überstürzten Passage der Keratinocyten durch die Epidermis zu verstehen. Tonofibrillen und Keratohyalin sind — trotz Vielschichtigkeit des Str. granulosum — vermindert. Die Hornschichten sind quantitativ wie qualitativ abnorm und in weiten Bereichen der Epidermis parakeratotisch. Es wird angenommen, daß der zugrundeliegende Gendefekt primär zur Proliferationssteigerung führt, aus der die weiteren Abweichungen von der normalen Verhornung resultieren. Die beim Sjögren-Larsson-Syndrom (Fall 4) vorliegenden Verhornungsstörungen stimmen nicht nur histopathologisch, sondern auch feinstrukturell mit denjenigen der I. congenita überein.
    Notes: Summary The ultrastructure of epidermal keratinization has been studied in 3 cases of ichthyosis congenita and in 1 case of Sjögren-Larsson syndrome. Corresponding to the increased rate of cell proliferation in these disorders, mitoses are observed more frequently in ultrathin sections. Ultrastructural findings at various stages of cell division suggest a normal mitotic course. Electron microscopically, there are quantitative changes in keratinization in the Malpighian layer. They are understood as a consequence of an accelerated keratinocyte transit through the epidermis. The quantity of tonofibrils is less than normal. Although the stratum granulosum is thickened, keratohyalin is diminished. In the horny layer hyperkeratosis and a marked degree of parakeratosis are present. It is assumed that a basic gene defect might primarily increase the cell proliferation, thus leading to alterations in keratinization. Corresponding to previous histopathological findings in Sjögren-Larsson syndrome keratinization on the ultrastructural level is the same as in ichthyosis congenita.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 243 (1972), S. 199-208 
    ISSN: 1432-069X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Intracelluläre Geißeln wurden innerhalb der menschlichen Epidermis an klinisch normaler Haut und bei verschiedenen Hauterkrankungen in Basalzellen und Melanocyten beobachtet. Die Anordnung der Mikrotubuli des Geißelzylinders entspricht mit einem 9 + 0-Muster dem Typ modifizierter Geißeln, die als sog. single cilia für zahlreiche Gewebetypen belegt sind. Aufbau und Feinstruktur der Geißeln werden beschrieben und mögliche Funktionen im Rahmen der Literatur diskutiert.
    Notes: Summary Intracellular cilia (single cilia) have been observed within basal keratinocytes and melanocytes of human epidermis in clinically normal skin and in several skin diseases. In transverse sections of cilia a central pair of microtubules characteristic of motile flagella is missing in the axoneme of the ciliary stalk. Thus by their 9 + 0 pattern of microtubule arrangement, intracellular cilia belong to the modified type of cilia. Ultrastructure and intrinsic features of intracellular cilia are discussed with reference to their possible function in epidermal cells.
    Type of Medium: Electronic Resource
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