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  • 1
    Electronic Resource
    Electronic Resource
    Ultrastructural study of the occurrence of autosomal dominant ichthyosis vulgaris in atopic eczema (1987)
    Springer
    Archives of dermatological research 279 (1987), S. 270-272 
    Details
    ISSN: 1432-069X
    Keywords: Atopic eczema ; Hyperlinear palms ; Autosomal dominant ichthyosis vulgaris ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00417327
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  • 2
    Electronic Resource
    Electronic Resource
    Disseminated cicatricial pemphigoid in a child and in an adult. Ultrastructural diagnostic criteria and differential diagnosis with special reference to acquired epidermolysis bullosa (1987)
    Springer
    Archives of dermatological research 279 (1987), S. 357-365 
    Details
    ISSN: 1432-069X
    Keywords: Disseminated cicatricial pemphigoid ; Acquired epidermolysis bullosa ; Dermoepidermal junction ; Initial blister formation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The first case of an infant affected with a rare, disseminated variant of benign cicatricial pemphigoid is described, showing the same ultrastructural features of initial blister formation as an adult patient. These consist in edematous changes within the superficial dermis caused by vesiculation or dissolution of cellular and noncellular connective tissue elements, coalescing into subepidermal blisters. Differential diagnosis excludes other nonhereditary bullous disorders because of the ultrastructure of the dermo-epidermal junction in nascent blisters and in perilesional skin. In spite of evident clinical, histological, and immunohistological similarities as well as controversial and confusing immunological studies, acquired epidermolysis bullosa can be clearly separated from our case by a diagnostic hallmark on the electron-microscopical level, i.e., band-like IgG depositions beneath the basal lamina. This is demonstrated in comparing the two cases of disseminated cicatricial pemphigoid with three patients suffering from acquired epidermolysis bullosa, thus providing evidence that disseminated cicatricial pemphigoid and acquired epidermolysis bullosa are two distinct nosologic entities.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00412620
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  • 3
    Electronic Resource
    Electronic Resource
    Disseminated bilateral hyperkeratotic variant of porokeratosis Mibelli (1987)
    Springer
    Archives of dermatological research 279 (1987), S. S38 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00585918
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  • 4
    Electronic Resource
    Electronic Resource
    Ichthyosis congenita type III (1988)
    Springer
    Archives of dermatological research 280 (1988), S. 268-278 
    Details
    ISSN: 1432-069X
    Keywords: Ichthyosis congenita ; Lamellar ichthyosis ; Keratinosome abnormalities
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We describe one type of the heterogeneous ichthyosis congenita group, inherited autosomal-recessively, noting its clinical and ultrastructural features based on the findings in a female patient, aged 30 at the time of first clinical and ultrastructural investigation, and supplemented with those of eight further patients, aged 2 to 22 years. Clinically this keratinization disorder was characterized by a generalized congenital ichthyosis with a reticulate skin pattern pronounced in a variable degree of severity, also involving the large flexures and the face, palms, and soles. Typical ultrastructural criteria were membrane structures, abnormal vesicular keratinosomes, vesicular complexes, and membrane-bound vacuoles within the cytoplasm of the granular cells, partly retained in the horny layer. A successful therapy with retinoids resulted in a complete removal of the hyperkeratoses but left the striking skin pattern unchanged. The morphological peculiarities remained unaltered as well. They are independent of the localization of the biopsies, of age and sex of the patients, and of oral and local treatment. Based on the clinical and ultrastructural features, this scaling disorder can be delineated against all other inherited ichthyoses and was termed ichthyosis congenita type III. A new nomenclature contributing to a distinct classification within the heterogeneous ichthyosis congenita group is discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00440599
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  • 5
    Electronic Resource
    Electronic Resource
    Acute bilateral renal vein thrombosis complicating Netherton syndrome (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 157-160 
    Details
    ISSN: 1432-1076
    Keywords: Key words Netherton syndrome ; Renal vein thrombosis ; Infant
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 1-year old male infant suffering from Netherton syndrome with severe generalized erythroderma presented with acute renal failure due to bilateral renal vein thrombosis (RVT) after a short episode of enteritis. The imperceptible fluid loss through the skin and the additional enteric water loss had led to decompensation of the delicate fluid balance and had resulted in RVT as a sequel of haemoconcentration. Reperfusion of the left kidney could be achieved by treatment with urokinase and heparin. Prophylactic oral anticoagulation was instituted for several weeks. Conclusion In severe Netherton disease meticulous surveillance of the fluid balance is important and aggressive treatment is indicated in case of additional fluid loss.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050789
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  • 6
    Electronic Resource
    Electronic Resource
    3H-Thymidine Labeling of Epidermis in Morbus Darier (1978)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of cutaneous pathology 5 (1978), S. 0 
    Details
    ISSN: 1600-0560
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: 3H-thymidine incorporation into epidermal cells of four patients with morbus Darier was studied by an in vitro technique. The labeling index counted on 0.5 μ thick plastic sections was 13.7 ± 5.3, indicating increased epidermopoieses in morbus Darier.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1600-0560.1978.tb00950.x
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  • 7
    Electronic Resource
    Electronic Resource
    Ultrastructure of Dyskeratosis in Morbus Darier (1977)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of cutaneous pathology 4 (1977), S. 0 
    Details
    ISSN: 1600-0560
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Biospy specimens from three patients with morbus Darier were studied by electron microscopy: Grains appeared in groups in the midst of dyskeratotic lesions and were recognized as being the result of more advanced acantholysis and precocious keratinization. Corps ronds were formed individually in the regions lateral to that of grains, where hypergranulosis was prominent in contrast to a mild dyskeratosis. Therefore, the formation of grains and corps ronds seem to be independent of each other. While in the literature acantholysis has mainly been ascribed to the separation of des-mosones into two segments, another initial process seems to be the primary disappearance of the attachment plaque along with the connecting tonofilaments. From the existence of the other contact complexes, it seems likely that a restoration of desmosomes between acantholytic cells should take place. In early stages of dyskeratotic cells, keratinosomes were increased in number and some of them persisted inside the plasma membrane without a release into the intercellular spaces, and thus they were present in grains and corps ronds. The dyskeratosis in morbus Darier is characterized by a premature, incomplete and individual keratinization as evidenced by the existence of keratohyalin granules and keratinosomes as early as at the level of the 3rd epidermal layer and the absence of a keratin pattern in the dyskeratotic horny cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1600-0560.1977.tb00905.x
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  • 8
    Electronic Resource
    Electronic Resource
    Keratin clumping in epidermolysis bullosa, Dowling-Meara type (1996)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 134 (1996), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1996.tb07868.x
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  • 9
    Electronic Resource
    Electronic Resource
    Electron microscopical evidence of unusual structures in the cytoplasm of some plasmotypes of Epilobium hybrids
    Amsterdam : Elsevier
    Journal of Ultrasructure Research 12 (1965), S. 624-633 
    Details
    ISSN: 0022-5320
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1016/S0022-5320(65)80052-1
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  • 10
    Electronic Resource
    Electronic Resource
    Besserung einer Ichthyosis congenita nach Varizellen-Infektion (1996)
    Springer
    Der Hautarzt 47 (1996), S. 869-870 
    Details
    ISSN: 1432-1173
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001050050525
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