摘要:

SUMMARYEpithelioma adenoides cysticum (EAC) is a well‐known genodermatosis which follows an autosomal dominant mode of inheritance. Questions still remain, however, concerning an apparent excess of females with EAC. An analysis of nineteen previously published pedigrees and one newly ascertained pedigree, which in the aggregate included over 175 cases of EAC, indicated no excess of affected females, but rather, a marked deficit of affected males. This deficiency was not the consequence of sex linkage or decreased viability, and was most evident in large pedigrees and sibships, sibships not including the probands, and late birth orders. In these situations, the penetrance of EAC in male gene carriers was only 50%, but was close to 100% in female gene carriers. Under maximum detection conditions, i.e. small pedigrees, small sibships, sibships containing the proband, and early birth orders, the penetrance in males increased to 85% and was again close to 100% in females. These findings suggested that the deficit of affected males was best explained by a combination of lessened expressivity and penetrance, effects which were magnified under situations of poor detection. A review of familial cases of EAC indicated no consistent associated anomalies such as those that characterize the naevoid basal cell carcinoma syndrom

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1976.tb07008.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

SUMMARYThis historical study has examined the persistent controversy about the propensity of epithelioma adenoides cysticum (EAC) to transform into multiple rodent ulcers, by reviewing cases reported through the years that seemed to support the idea. After focusing on the biological behaviour and natural history of the tumours and comparing them with our present knowledge of the behaviour of EAC, we believe that these cases were incorrectly diagnosed. Rather than EAC, hey were probably examples of the naevoid basal cell carcinoma syndrome. The latter syndrome was actually established by the recognition that patients who have multiple rodent ulcers that appear early in life and behave aggressively usually have a constellation of associated developmental defects and that the tumours should be classified as genetically determined basal cell carcinomas and not as EAC.

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1976.tb07009.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

SUMMARYLesions of erythema multiforme from seventy‐five patients have been studied histologically. In addition to peculiar intercellular epidermal oedema, subepidermal separation and a Lymphohistiocytic inflammatory infiltrate in the papillary dermis, epidermal cell necrosis was observed in a variable percentage of the lesions. While dermal disturbance was a predominant finding in the macular lesions, focal or generalized keratinocytic necrosis was seen in the macular, papular, bullous and iris lesions in that order of frequency. Significant numbers of eosinophils were present in the inflammatory infiltrate in 60% of the bullous and 28% of the macular lesions. Our findings suggest that erythema multiforme represents a tissue reaction with spectral expression, one end presenting as a predominantly dermal disturbance and the other merging into the adult type of toxic epidermal necrolysis. Yet, the histological features remain sufficiently characteristic for differentiation from other erythematous and vesiculobullous eruption

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1976.tb07010.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

SUMMARYThree cases of secondary syphilis are described in whom a skin biopsy was performed. In all, the histology bore a striking resemblance to that of malignant lymphoid neoplasm.The classical cutaneous manifestations of secondary syphilis are usually easy to diagnose, especially when there is a good clinical history and the appropriate investigations are positive. If, however, the eruption is not characteristic, a skin biopsy may be carried out. We should like to report three cases where the histological appearances in isolation would have been misleading.

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1976.tb07011.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

SUMMARYThe importance of various unsaturated fatty acid triglycerides to the repair of faulty skin barrier function was studied in essential fatty acid‐deficient rats. Following cutaneous application of the pure triglycerides for up to 5 days, the hitherto high rate of transepidermal water loss, characteristic of essential fatty acid deficiency in rats, was reduced by the triglycerides of linoleic and γ‐linolenic acids. Incorporation of the applied fatty acids into the lecithin of the epidermis accompanied these changes in water loss, indicating that cutaneously applied triglycerides may be metabolized by the skin and incorporated into complex lipids. Other fatty acid triglycerides, including α‐Unolenic, dihomo γ linolenic, arachidonic and ω‐7‐heneicosatrienoic acid, did not lower the rate of ransepidermal water loss, although all were incorporated into epidermal structural lipids. The non‐essential oleic acid also had no effect upon the rate of transepidermal water loss. These data suggest that of the two main essential fatty acids that occur in skin, linoleic acid and arachidonic acid, the former specifically plays an important role in regulating barrier function whereas the latter may have a separate function, such as serving as a precursor of

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1976.tb07012.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

SUMMARYEffective diffusion constants have been determined for the permeation of nandrolone through whole and stripped human abdominal cadaver skin in vitro. From the values obtained, the diffusion constant for nandrolone in the stratum corneum has been inferred. The experimental approach used yields reproducible results for skin samples taken from a single individual. There is also a surprising degree of uniformity in the values obtained for skin from different individuals.

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1976.tb07013.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

SUMMARYThe activities of 14 acid hydrolases have been measured in normal and diseased human epidermis. Our findings were as follows: (i) Increased hydrolase activity was common in lesions; the clinically uninvolved skin of patients, however, invariably showed normal values. (2) The pattern of change was independent of the nature of the disease, the arylsulphatases and β‐glucosidase usually showing the greatest elevations. (3) Themagnitudeof the changes differed from disease to disease, being most marked in the lesions of psoriasis. (4) Ichthyosiform erythroderma was exceptional in showing elevated levels of the galactosidases and cathepsins B, and D. We suggest that (with the exception of ichthyosiform erythroderma) the abnormalities which we have observed may be related to the proliferative rate of the epiderm

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1976.tb07014.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

SUMMARYThe distribution and excretion of the staphylococcal exfoliatin was investigated following in vivo administration of highly purified125I‐labelled exfoliatin fractions to adult and newborn mice. Adult mice excrete approximately one‐third of a test dose by 3 hours as compared to a fifteenth of a test dose excreted by newborn mice. Accordingly, blood tracer radioactivity reaches a relatively higher peak and shows a slower decline in newborns than in adults. The urine of adult mice contains considerable biologically active exfoliating material. Both nephrectomized and carbon tetrachloride‐poisoned adult mice injected with exfoliatin develop generalized exfoliation whereas comparable doses in untreated controls have no effect. On the other hand, subtotal hepatectomy, followed by injection of exfoliatin, does not lead to exfoliation. We conclude that renal immaturity is a critical factor responsible for the susceptibility of neonates to generalized staphylococcal scalded skin syn

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1976.tb07015.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

SUMMARYThe amounts of agglutinating antibody to staphylococcal and micrococcal phenol‐extracted (probably teichoic acids) and protein antigens, prepared from Baird Parker types SI, SIV and Ml, were no greater in eczematous than in control persons. Most antibody to phenol‐extracted antigens was IgM which frequently activated complement to lyse red cells which had adsorbed the bacterial antigen. IgG antibody against phenol‐extracted staphylococcal antigen (Staph. aureus, Baird Parker type SI) was shown to be specific antibody combining by the F(ab)2 portion of the molecule. The phenol‐extracts did not contain staphylococcal Protein A that binds to the Fc portion of IgG. Furthermore, the presence of Protein A in the protein or total antigen extracts did not appear to modify the results of the tanned cell agglutination test.The occurrence of immediate or of 4 h, Arthus‐like skin test responses to staphylococcal or micrococcal antigens was unrelated to the agglutinin or complement‐lysis titre of the releva

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1976.tb07016.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

SUMMARYWe have investigated lymphocyte subpopulations (six different markers) and T cell functions (mitogen responses and serum thymic factor determination) in twenty patients with psoriasis compared with thirty‐five healthy subjects. Normal results were found for B cell markers, but significantly lower numbers of T cells were assessed by E‐rosettes and anti HTLA serum. Mitogen responses were normal for PHA but depressed after Con A and PWM stimulation. Lastly, thymic factor levels were significantly higher in psoriasis than in normal subjects of the same age.These findings could be in favour of a T cell subpopulation defect involved in an auto‐immune pathogenesis of psor

ISSN:0007-0963    

DOI:10.1111/j.1365-2133.1976.tb07017.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY