摘要:

Abstract:Disturbances in the process of normal cornification leading to pathologic scaling provide the pathophysiologic basis for the ichthyoses. These disturbances may result from either abnormalities in protein metabolism (keratinization) (i.e., the “bricks”) or in lipid metabolism (i.e., the “mortar”) (Fig. 1). The evidence linking the various ichthyoses to defects in protein or lipid metabolism have been reviewed. It is likely that future advances will lead not only to a more complete understanding of the pathogenesis of these disorders, but also will shed significant light on the normal stratum corneum functions of barrier formation and desquamation, as well as lead the way to more rational and effective therapies. In recent years, prenatal diagnosis has been successfully performed in several of the ichthyoses. It is likely that improvements in our ability to prenatally diagnose those disorders will advance hand‐in‐hand with further progress in unraveling their underl

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1983.tb01087.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Abstract:Tyrosinemia II is caused by a deficiency of hepatic tyrosine amino‐transferase. With the deficiency of this key enzyme of tyrosinc catabolism there is an increase in plasma tyrosine and then an increase in tyrosinc metabolites in the urine. The increased plasma tyrosinc causes tyrosine to crystallize in the cornea, producing corneal ulcerations and sometimes proliferation of corneal epithelium. In the epiderm is of the palms and soles, tyrosine leads to erosions, crusting, and then hyperkeratosis. The human disease is due to an autosomal recessive gene, and similar genetic diseases have been found in mink and in dogs. A nutritional model for the disease, in which a high‐tyrosine low‐protein diet is ted to rats, produces almost identical features. The features of this disorder and some of the implications of this disease for the study of other genetic diseases is discussed in this r

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1983.tb01088.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Abstract:A variety of skin diseases have been reported to have associated abnormalities in PMN chemotactic motility; however, the relationship of abnormal leukocyte motility to thc pathogenesis of these diseases is poorly understood. A common feature in skin diseases associated with depressed chemotaxis has been the frequency of recurrent pyogenic infections. Since the chemotaxins and methodologies for evaluating leukocyte chemotaxis differ among laboratories, little is knownabout the specificity of these chemotactic defects. Further information regarding PMN motility defects may be obtained from detailed studies of migration to various doses of chemotaxins. Thecommercial availability of potent, structurally defined chemotaxins, sueh a N‐formylatcd pcptides, provides laboratories the opportunity to evaluate migration to “common” chemotaxins. In addition, the development of the multiple microwell chemotaxis chamber has made possible the opportunity to collect substantial leukocyte motility data from relatively small blood samples. Further investigations of leukocyte motility in various dermatologic diseases are clearly needed, Amore systematic approaeh, i.e, (1) use of several different chemotaxins at various doses in chemotaxis assays and (2) use of standard, structurally defined chemotaxins. would facilitate com‐parative analyses of elinical studies among laboratories. It is likely that this approach would provide more specific information about the relevance of leukocyte motility defects in these d

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1983.tb01089.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Abstract:Tumors make up almost 25% of the causes of cutis verticis gyrata. An underlying dermal nevus or neurofibroma is the tumor most often found. Two new cases of cutis verticis gyrata caused by an intradermal nevus cell nevus (cerebriform intradermal nevus) are presented. The importance of early diagnosis and aggressive surgical excision and plastic reconstruction in an attempt to minimize the emotional trauma of the required surgery is stressed. Cerebriform intradermal nevi are a type of large congenital nevi and should be evaluated with this in mind.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1983.tb01090.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Abstract:Two children with pili torti (twisted hairs) are reported, one of whom had sensorineural hearing loss. This combination of pili torti and deafness was described as a new genetic entity by Björnstad in 1965. Although light microscopy demonstrated variable numbers of twists along the shafts of hairs obtained from both patients, scanning electron microscopy in both cases revealed flattening and diagonally arranged scalloped defects on only one side of the involved hair shafts rather than true twists. Early auditory testing should be performed in children with pili torti in order to secure early treatment of possible associated hearing loss

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1983.tb01091.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Abstract:In this review of the basie anatomy and embryology of thc human eyelids the sequence of eyelid differentiation is discussed. The development is important in exploring thc formation of divided or “kissing” nevi of the lids. Since the lids are fused from the ninth week until the twentieth week of gestation one may propose that the precursor elements of the nevus develop during that specific time. Because the epithelium only is fused, melanocytes derived from the neural crest must begin their differentiation in the region of the fused epithel

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1983.tb01092.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Abstract:An unsclectcd cohort of 4,641 newboms was ascertained prospectively for the purpose of detecting any cutaneous lesion. These were catalogued into pigmented lesions, vascular lesions, and miscellaneous lesions. Several important findings were elucidated: congenital nevocellular nevi are speckled at their borders: no white newborn in the study had more than one café au lait mark; a hypopigmented tuft of hair was seen in one infant with tuberous sclerosis but is found more commonly in normal individuals; a previously undescribed lesion called zosteriform melanocytic nevus was seen as a normal pigmentary variant in blacks; and hypopigmented macules seen at birth are seen primarily in normal infants. It is hoped that these findings will allow the pediatrician and dermatologist to offer more meaningful prognostic information to their patients

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1983.tb01093.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Abstract:To summarize the quantity, quality, and subject matter of articles pertaining to pediatric dermatology in current pediatric and dermatologic journals. 2,010 articles published in 1981 in 9 clinical journals were reviewed. Of articles in the pediatric journals, 4% had a primary dermatologic focus, and another 6% had a secondary dermatologic focus. Of the clinical articles in the dermatologic journals, 15% concerned only pediatric patients and an additional 18% involved both pediatric and adult patients. The great majority of pediatric (65%) and dermatologic (83%) articles concerning pediatric dermatology were case reports, with lesser proportions of research reports and review articles. There was a relative scarcity of prospective clinical studies describing either the natural course of pediatric skin diseases or the effects ot various therapies upon these diseases. Frequent topics of articles in both types of specialty journals included cutaneous manifestations of systemic disease, hereditary skin disorders, and bacterial skin infections. It is hoped that interest in this important field will promote mutually beneficial collaboration between pediatricians and dermatologists in the areas of patient care and clinical research.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1983.tb01094.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1983.tb01095.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1983.tb01096.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY