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  • 1990-1994  (8)
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Material
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Year
  • 1
    Electronic Resource
    Electronic Resource
    Expression of mutant p53 gene in squamous carcinoma arising in patients with recessive dystrophic epidermolysis bullosa (1992)
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 20 (1992), S. 0 
    Details
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: J.A.M. is currently receiving a research fellowship from the Dystrophic Epidermolysis Bullosa Research Association (DEBRA).Epidermolysis bullosa, a rare genodermatosis, is characterized by increased skin fragility manifest as blistering and sometimes accompanied by scarring. The latter is particularly severe in the recessive dystrophic variant and may be complicated by the development of squamous carcinoma in up to 30% of patients. We have studied 23 such tumours in six patients with this variant, with an anti-serum to p53 protein. Twenty-six per cent of the squamous carcinomas labelled positively for mutant-type p53 protein. This low figure, however, reflects the large number of well-differentiated tumours in this series, where 14 out of 15 were negative. In the moderate to poorly differentiated examples the positivity rate was 63%. Of the three patients in the latter category, one has died from disseminated tumour and another has widespread metastases. The findings support the hypothesis that mutant p53 protein expression correlates with poorer tumour differentiation. They also suggest a possible correlation between p53 protein expression and tumour behaviour.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2559.1992.tb00962.x
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Fibrillin immunoreactivity is associated with normal or fragmented elastic microfibrils at the dermal-epidermal junction in recessive dystrophic epidermolysis bullosa (1994)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 131 (1994), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Elastic microfibrils containing fibrillin are a part of the fibroreticular network of normal epidermal basement membrane. In dystrophic epidermolysis bullosa (DEB) at least one other fibroreticular component, anchoring fibrils, which contain type VII collagen, is known to be abnormal. We therefore questioned whether elastic microfibrils and fibrillin expression might also be abnormal in DEB. By indirect immunofluorescence, and pre-embedding immunogold electron microscopy using a monoclonal antifibrillin antibody, we found no difference from control samples in either the quantity of the labelling or in the ultrastructural appearances of the immunolabelled fibrils in intact DEB skin. In areas of dermal-epidermal separation, however, we observed a number of thin, fragmented sublamina densa wisp-like structures, which still labelled for fibrillin despite lacking the typical ultrastructural features of normal elastic microfibril bundles. As a consequence of blistering in DEB, elastic microfibril bundles are disrupted, and fragmented microfibrils may still remain attached to the blister roofs. Many of these elastic microfibrils cannot be distinguished from rudimentary or altered anchoring fibrils on morphology alone, and might therefore account for misinterpretation of ultrastructural disorders of the dermal-epidermal junction. We postulate that, in intact skin, elastic microfibrils might contribute to dermal-epidermal adherence, in the absence of normalfunctioning anchoring fibrils.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1994.tb08545.x
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Epidermolysis bullosa simplex (Dowling-Meara). A clinicopathological review (1992)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 126 (1992), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The clinicopathological features of 22 cases of the Dowling-Meara form of epidermolysis bullosa simplex (DM-EBS) (11 males. 11 females: aged 5 days-46 years) were reviewed using data collected over a 10-year period. All cases presented clinically within the first 5 days of life. Early blisters were often large (up to 5 cm in diameter), and were mostly acral and particularly periungual. Some cases presented with more widespread erosive skin changes, and two neonates with extensive skin involvement died as a result of overwhelming sepsis. After the neonatal period a different pattern of blistering occurred with more proximal haemorrhagic, herpetiform clusters of blisters. Central healing with recurrent blistering at the margins of these areas was frequently noted. Other physical signs included varying degrees of intra-oral blistering, nail shedding, nail dystrophy, minor scarring, palmo-plantar keratoderma, a lack of seasonal variation and improvement during later childhood. The underlying pathological mechanism in DM-EBS is basal cell cytolysis, or rarely acantholysis, in association with tonofilament (TF) clumping. TF clumping was found in lesional, perilesional and some non-lesional skin, suggesting that the tonofilament abnormality may be of primary aetiological significance in DM-EBS. TF clumping may be due to specific keratin abnormalities because the altered TF were found in a distribution similar to the known distribution of the basal cell keratins. K5 and K14. The level of blistering was invariably very low within the epidermal basal layer and often less than 0·5 μm above the basement membrane. We conclude that DM-EBS is a distinct, and probably under-recognized genodermatosis which tends to have a good prognosis. However, the disease can occasionally be severe, especially during the neonatal period, when it may be confused with junctional or severe recessive dystrophic EB. Electron microscopy is the best means for demonstrating the characteristic cytoskeletal disorder and confirming the diagnosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1992.tb11813.x
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  • 4
    Electronic Resource
    Electronic Resource
    (3) Recessive dystrophic epidermolysis bullosa and multiple primary squamous cell carcinomata (1991)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 125 (1991), 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.1365-2133.1991.tb05475.x
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  • 5
    Electronic Resource
    Electronic Resource
    Epidermolysis bullosa pruriginosa: dystrophic epidermolysis bullosa with distinctive clinicopathological features (1994)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 130 (1994), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary We report a study of eight unrelated adult patients with a highly distinctive phenotype of dystrophic epidermolysis bullosa. It is characterized clinically by pruritus, lichenified or nodular prurigo-like lesions, violaceous linear scarring, occasional trauma-induced blistering, excoriations, milia, Nail dystrophy and, in some cases, albopapuloid lesions on the trunk, The scarring is most evident on the limbs, particularly on the shins, with relative sparing elsewhere. Intact blisters are rarely seen. Physical signs were present at birth in three patients, but in the others skin manifestations were first noticed between 6 months and 10 years of age. Five cases are sporadic, but three of the eight patients have a history of familial involvement, with autosomal dominant inheritance in two cases and recessive transmission in the other case. Studies of the dermal-epidermal junction showed alterations in the number and ultrastructure of anchoring fibrils in lesional, perilesional and non-lesional skin, consistent with a diagnosis of dominant or localized recessive dystrophic epidermolysis bullosa. These patients represent an unusual, poorly recognized form or expression of dystrophic epidermolysis bullosa which has features in common with a variety of acquired inflammatory dermatoses.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1994.tb13109.x
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  • 6
    Electronic Resource
    Electronic Resource
    The presence of autoantibody to recombinant lipocortin-I in patients with psoriasis and psoriatic arthritis (1990)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 123 (1990), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Corticosteroids may mediate some of their anti-inflammatory effects via induction of a specific 38 kDa protein, lipocortin-I. Anti-lipocortin-I antibodies (ALA) were measured by enzymelinked immunosorbent assay (ELISA) in 23 patients with plaque-type psoriasis alone (NAP), in 21 patients with psoriasis and arthritis (PA), and in 67 healthy controls. Only two of 23 NAP patients had elevated ALA, whereas six of 21 PA patients had raised levels of ALA (P= 0.2). Sero-negative polyarthritis was the most common pattern of joint disease in those PA patients with elevated ALA (4/6). ALA levels did not correlate with the extent or severity of cutaneous involvement, and are unlikely to be involved in the pathogenesis of cutaneous psoriasis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1990.tb01472.x
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  • 7
    Electronic Resource
    Electronic Resource
    Aquagenic pruritus and the myelodysplastic syndrome (1990)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 123 (1990), 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.1365-2133.1990.tb06305.x
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    Paper (German National Licenses)
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  • 8
    Electronic Resource
    Electronic Resource
    Intracellular expression of type VII collagen during wound healing in severe recessive dystrophic epidermolysis bullosa and normal human skin (1992)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 127 (1992), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary The ability of keratinocytes to synthesize basement membrane components in vivo during wound healing in normal human skin and in severe recessive dystrophic epidermolysis bullosa (RDEB) was investigated. Indirect immunofluorescence using anti-type VII collagen (VIIc, recognizing the globular non-helical component of the molecule), anti-type IV collagen, anti-laminin and bullous pemphigoid antisera, was performed on biopsies of intact skin and of healing skin taken between 7 and 14 days after dermatome injury (upper to mid-dermal wounding) in eight patients with severe RDEB and in seven normal subjects. Baseline anti-type VIIc immunofluorescence showed completely absent staining of the epidermis, dermis and dermo-epidermal junction in severe RDEB samples, and bright linear dermo-epidermal junction fluorescence in normal human skin. In 5/5 normal human skin samples taken 9–12 days post-wounding, some type VIIc expression was noted within basal cells as well as in a continuous or interrupted linear distribution at the basement membrane zone. In all the severe RDEB biopsies sampled between days 10 and 13 (5/5), anti-VIIc fluorescence was also seen with varying intensity within basal and lowermost suprabasal cells, and in one day 14 sample at the dermo-epidermal junction. Low levels of intracellular type IVc were seen in both groups, but only in those samples taken 7–9 days after injury; later biopsies showed only continuous dermo-epidermal junction staining. Linear basement membrane zone labelling with laminin and bullous pemphigoid antisera was seen in all samples in both sets of subjects, even at day 7, but there was no detectable intracellular antisera staining. The finding of anti-type VII collagen immunofluorescence staining within the lower epidermis in severe RDEB during wound healing implies that these keratinocytes, under certain circumstances, have an inherent capacity to synthesize type VIIc, or at least part of the non-collagenous globular region of the molecule, and that a complete absence of type VIIc is not the primary abnormality in the most severe form of RDEB.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1992.tb00447.x
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