摘要:

SummaryThe 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 c

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1992.tb11813.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

SummarySkin biopsies were taken from 11 patients with morphoea, nine with acrosclerosis and 10 with diffuse systemic sclerosis and processed for immunohistochemical studies using a panel of monoclonal antibodies including antibodies to MHC class II antigens. A significantly higher percentage of HLA‐DR positive dermal cells were observed in the reticular dermis in biopsies from patients with morphoea (44·1 ± 16·2%). acrosclerosis (15·9 ± 5·4%) and systemic sclerosis (39·5 ± 2·3%) when compared with the controls (6·6 ± 2%), A smaller percentage of dermal cells also expressed HLA‐DP and ‐DQ. The degree of mononuclear cell infiltrate in the biopsies, however, did not correlate with the percentage of HLA class II positive fibroblasts. In organ culture, the expression of the HLA class II antigens was almost totally lost after 3 days and was no longer detected on fibroblasts after 3

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1992.tb15112.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

SummaryIntravital capillaroscopy using a video‐microscopy system permits real‐time imaging of the skin microvasculature with retrospective analysis of capillary dynamics. The addition of fluorescein angiography improves contrast and detects aspects of blood vessel behaviour, such as perfusion homogeneity and transcapillary solute diffusion, not detectable under native conditions. This study was performed to evaluate whether the method can be applied to the investigation of a skin disease and in particular the understanding of the role of the blood vessel in the pathogenesis of psoriasis. Results demonstrated clear differences between normal and psoriatic skin. More capillaries were red cell perfused in both plaque and uninvolved skin compared to normal skin (PP<0·02. respectively). The capillaries in psoriatic plaque skin were much larger than those in normal skin (P<0·001). The density of capillaries was not increased in plaque or uninvolved psoriatic skin, indicating expansion of existing vessels and not new vessel formation. The area of fluorescence seen around each capillary at 60 s was greater in plaque (P<0·001) and in uninvolved psoriatic skin (P<0·001) than in normal skin, indicating greater vessel transcapillary diffusion. This study confirms the value of video‐microscopy as a non‐invasive technique for the examination of the cutaneous microcircu

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1992.tb15113.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

SummaryTo estimate the prevalence of the subgroups of porphyria cutanea tarda (PCT), erythrocyte uroporphyrinogen decarboxylase (UD) activity was measured in 80 unrelated patients with PCT, and in 45 of their relatives by using pentacarboxyl‐porphyrinogen III as substrate. The subgroups were differentiated by analysis of the urinary porphyrins of the patients and 119 of their relatives. Of the patients, 77·5% were found to be suffering from the sporadic form of PCT (type I PCT), and 22·5% from the familial form (type II PCT), Every patient with PCT had previously been affected by alcohol, oestrogen or some other liver‐damaging factor. The relative frequency of familial PCT was higher in females (nine of 15) than in males (nine of 65), which suggests that inheritance of the gene for type II PCT may predispose to oestrogen‐precipitated PCT. The onset of type II PCT occurred at a lower age than that of type I (42·6 vs. 47·0 years). The findings suggest an increased risk of precipitating factors in carriers of an inherited UD d

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1992.tb15114.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

SummaryIn an investigation of the role of keratinocytes in the pre‐immunological phase of contract allergy, we have studied the effect parphenylendiamine (PPD) on cell proliferation, membrane lipid peroxidation and the expression of the intercellular adhesion molecule 1 (ICAM‐1). Because PPD undergoes rapid autoxidation in the culture medium, the effect of PPD‐modified medium on keratinocyte proliferation and ICAM‐l expression was also examined.PPD at low concentrations (up to 10 μg/ml) and with low exposure times (0·5 h) enhanced keratinocyte proliferation, but at high concentrations and with longer exposure times resulted in cell stasis and toxicity. These effects and the enhanced membrane lipid peroxidation that was also observed can be ascribed to the production of superoxide and hydrogen peroxide by the autoxidation of PPD in the medium. At non‐cytotoxic concentrations, PPD induced ICAM‐1 expression on the keratinocytes. PPD‐modified medium was also cytotoxic to the keratinoeytes and induced ICAM‐1 expression in non‐cytotoxic concentrations. It appeared that superoxide and hydrogen peroxide were not responsible for the cytotoxicity. These results are consistent with the view that oxidative stress may be an essential part of the pre‐immunological phase in the induction of allerg

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1992.tb15115.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

SummaryThe peripheral T‐cell lymphomas represent a heterogeneous group and include, besides mycosis fungoides and Sézary syndrome, large‐cell anaplastic lymphoma. We report 1 S cases from our files I that fulfil the histological criteria of pleomorphic T‐cell lympoma with primary skin involvement. Most of the cases were elderly with a male‐to‐female ratio of 1·5: 1. The HTLV‐1 serology was negative. The clinical features of these patients differed from those with mycosis fungoides and Sezary syndrome, in that eczematous and precursor lesions such as parupsoriasis en plaque were lacking apart from one exception. All the patients with small‐cell pleomorphic T‐cell lymphomas were alive, although three of the nine patients with medium‐to‐large tumour cells have died. Pleomorphic T‐cell lymphoma should be regarded as a distinct entity among the lymphoproliferativ

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1992.tb15116.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

SummaryAberrant expression of class 11 products of the major histocompatibility complex (HLA‐D locus antigens) occurs on keratinocytes in several inflammatory dermatoses and on thyroid epithelial cells in autoimmune thyroiditis. The functional significance of aberrant HLA‐D expression is unclear but it has been hypothesized that epithelial cells bearing these determinants may act as antigen‐presenting cells for autoantigens. The aim of the present study was to investigate the pathogenesis of bullous pemphigoid using immunohistotochemical methods to determine whether the HIA‐D locus antigens are aberrantly expressed on keratinocytes in lesional and uninvolved skin. A panel of monoclonal antibodies to each of the HLA‐D subregions (DR, DP and DQ) and to Langerhans cells was used. Epidermat expression of the HLA‐D locus antigens was similar in patients and controls, and there was no significant increase in expression in lesional skin compared with uninvolved skin in six out of nine patients. In three out of nine patients slight enhancement of epidermal HLA‐D expression in lesional epidermis corresponded to increased Langerhans cells rather than expression on keratinocytes. HLAD locus antigens are absent from keratinocytes in bullous pemphigoid skin and aberrant expression of these determinants cannot therefore be implicated in antigen

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1992.tb15117.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

SummaryBullous pemphigoid (BP) is characterized by the production of autoantibodies against BP antigens, γ‐interferon (γ‐IFN), a T‐cell lymphokine, is known to enhance the expression of several cell‐surface proteins. In this study, keratinocytes were cultured in the presence of γ‐IFN. the expression of BP antigen protein was examined by flow cytometry and BP antigen messenger RNA (mRNA) (encoding 230‐kDa protein) was quantified by slot‐blot hybridization. The results indicated that BP antigen gene expression by keratinocytes was upregulated by γ‐IFN. This enhancement of gene expression was I detected at both the protein and mRNA level, suggesting pretranslational regulation. These results imply the involvement of not only humoral immunity but also cell‐mediated immunity in

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1992.tb15118.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

SummarySince 1981, all pregnant women presenting to our department with a pruritic dermatosis have been investigated by histological and immunopathological techniques. We recruited 111 patients and performed skin histology in 77. 109 direct immunofluorescence tests (DIF), 74 indirect immunofluorescence tests (IIF) and 15 Western blots (WB).We identified: (i) five typical cases of pemphigoid gestationis (PG) (4·5%), corresponding to an incidence of 1/7000 pregnancies, (ii) Five cases without PG but showing circulating anti‐BMZ antibodies of IgM type. With the exception of one case, clinical features were homogeneous‐occurrence of erythematous papular and/or urticarial lesions on the trunk, and less often, on the limbs between the 32nd and 38th week of pregnancy. Rapid clearance of lesions within a few days was the rule. Whenever performed, DIF was negative and IIF showed circulating anti‐BMZ antibodies of IgM type. Western blot studies were negative in these five cases, (iii) One hundred and one cases with negative immunofluorescence tests, considered to be suffering from polymorphic eruption of pregnancy.Our results show the value of systematic immunopathological investigations in pregnant women presenting with a pruritic dermatosis, and raise the possibility of a new entity, as defined by circulating anti‐BMZ antibodies of

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1992.tb15119.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

SummaryThe extracellular matrix of the hair follicle dermal papilla is rich in glycosaminoglycans, the expression of which varies during the hair growth cycle being maximal in anagen and becoming undetectable as the follicle enters telogen. These observations, together with other experimental and clinical evidence, suggest that glycosaminoglycans may be involved in regulating hair growth. To investigate the metabolism of glycosaminoglycans by the dermal papilla we have measured the incorporation of radiolabelled precursors into glycosaminoglycans released into extracellular matrix and culture medium by cultured human dermal papilla cells. We also studied glycosaminoglycan synthesis by cells cultured from the lower follicular connective tissue sheath and by non‐follicular dermal fibroblasts. Compared with dermal fibroblasts, dermal papilla cells showed a three to fourfold higher level of incorporation of35S‐sulphate and3H‐glucosamine into extracellular matrix glycosaminoglycans. Dermal papilla cells also released more3H‐glucosamine‐labelled glycosaminoglycan into culture medium than dermal fibroblasts but there was no difference in35S‐sulphate labelling. These findings indicate that dermal papilla cells maintain a high level of glycosaminoglycan synthesisin vitro.Specific enzyme/chemical degradation showed that dermal papilla cells and dermal fibroblasts synthesized the same glycosaminoglycan types. However, the results suggested that dermal papilla glycosaminoglycans are less sulphated than those synthesized by dermal fibroblasts and that a higher proportion of sulphated glycosaminoglycans is retained in an extracellular matrix. The synthesis of glycosaminoglycans by connective tissue sheath cells was similar to that of dermal papilla cells, supporting the view that the dermal papilla and connective tissue sheath share certain

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1992.tb15120.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY