摘要:

The aim of diagnosis is the characterization of a patient's disease so that the optimal therapy may be instituted. The precision of the diagnosis depends on the knowledge available and the sensitivity of the techniques used to exploit that knowledge, and major methodological advances in recent years have provided the scientific investigator with an extensive array of sophisticated techniques. The choice of which test is best for the diagnosis of a particular disease is particularly difficult when dealing with the disorders of porphyrin metabolism as simple, cheap tests are available to identify a patient as “porphyric.” This diagnosis is not sufficient, however, as the term “porphyria” encompasses a number of different disorders, each a manifestation of deficient enzyme activity at different stages in the heme biosynthetic pathway. While all are characterized by excessive production, accumulation, and excretion of porphyrins and porphyrin precursors, the pattern thereof is specific for each particular type of porphyria and each is a discrete clinical entity demanding a different therapeutic approach. It thus cannot be over emphasized that the porphyric patient's disease must be meticulously defined and when simple tests are insufficient for this, sophisticated techniques should be employed, preferably in laboratories committed to porphyrin study. Before discussing the best way to make a diagnosis, however, it is clearly important to define the ter

ISSN:0011-9059    

DOI:10.1111/j.1365-4362.1984.tb05656.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

ISSN:0011-9059    

DOI:10.1111/j.1365-4362.1984.tb05657.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

ISSN:0011-9059    

DOI:10.1111/j.1365-4362.1984.tb05658.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

The insidious intrusions of arbitrary restrictive patterns of health care delivev that limit direct access of patients to specialist care were once largely confined in this country to voluntary health maintenance organization‐independent practice association (HMO‐IPA) systems and the military service. In 1981, 87% of surveyed HMOs and IPAs restricted patient access to specialist care to some extent. Such limitation is now becoming more widespread through the creation of primary‐care networks, case management, and experimental programs under Medicaid. Limitation of direct access to specialists will impact substantially on the practice patterns and quality of dermatologic care, because primary care physicians and physician extenders who will control the referral system often will attempt to treat skin conditions even though they frequently misdiagnose the condition. Further, as is often the case, referrals to specialists often will be limited to one visit only, with no opportunity for the specialist to establish a satisfying doctor‐patient relationship or to evaluate the effectiveness of treatment. The following is an analysis of this phenomenon and an assessment of what has been accomplished or remains to be done to combat thi

ISSN:0011-9059    

DOI:10.1111/j.1365-4362.1984.tb05659.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

Were we to discuss the cause of acne a hundred years ago, it would be quite simple. Louis Duhring,Pathfinder for American Dermatology, considered the etiologies of acne. They “are numerous and are very different in their nature…. The most frequent and ordinary origin of acne is puberty…. [the] general disability of the system occupies a conspicuous place. Under this head may be grouped all those abnormal conditions arising from imperfect physical development, improper nutrition, and other defects of the economy, whether organic or functional.” So much for the 19th

ISSN:0011-9059    

DOI:10.1111/j.1365-4362.1984.tb05660.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

ABSTRACT:The distribution of S100 protein in normal skin and various tumors involving skin was assessed using rabbit antibody to S100 protein in an immunoperoxidase reaction. In normal skin, S100 was detected in the epidermis (melanocytes and Langerhans' cells), dermis (Schwann cells, Pacinian and Meissner's corpuscles, and interdigitating reticulum cells), cells of the sweat gland apparatus, and in chondrocytes. In tumors involving skin, S100 protein was present in nevi, malignant melanomas, histiocytosis X, mixed sweat gland tumors, neural tumors, chondromas, and chondrosarcomas. Detection of S100 protein by immunostaining was useful in understanding the histogenesis of various skin tumors and in assessing the diagnosis and prognosis of a variety of skin lesions encountered in surgical pathology.

ISSN:0011-9059    

DOI:10.1111/j.1365-4362.1984.tb05661.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

ABSTRACT:Variably acid‐fast cocoid forms, suggestive of cell wall deficient forms of mycobacteria, were observed in the dermis in microscopic sections of skin from six patients with generalized scleroderma, 10 patients with localized scleroderma (morphea), and four patients with lichen sclerosus et atrophicus(LSA). These cocoid forms werde found within the collagen bundles, around the adnexae (Hair shafts, pilosebaceous units, eccrine glands), and less commonly around the blood vessels and nerves. These cocoid forms may be related to cocci and also to granular cocoid elements of corynebacteria‐like coccobacilli, which, on occasion, can be cultured from the skin of these three diseases. The findings in this study support the three‐decade old hypothesis concerning the constant association of pleomorphic acid‐fast bacteria with scleroderma. The study also suggests that closely related diseases, such as morphea and LSA, are also associated with the presence of similar appearing m

ISSN:0011-9059    

DOI:10.1111/j.1365-4362.1984.tb05662.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

ABSTRACT:Tissue typing performed on the lymphocytes of 41 Patients, including: 23 patients with ichthyosis (15 of autosomal dominant and 8 of the x‐linded variety; 8 patients with tuberous sclerosis (epiloia); and 10 patients with acroder‐matitis enteropathica (AEP). In adddition, tissue typing was performed for a control group of 80 healthy unrelated subjects of the same ethnic origin as the patients. A significant increase of A2antigen or its crossreacting antigen A28was detected among the three diseases studied. A significant increase of HLA‐A2antigen was found in autosomal dominant ichthyosis, while the x‐linked variety showed significant association with HLA‐Cw2. A significant association with HLA‐A28antigen was noticed in both epiloia and AEP. The suggested mechanisms of such association may be refferred to as incidental genetic linkage with mutants causing disease or

ISSN:0011-9059    

DOI:10.1111/j.1365-4362.1984.tb05663.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

ABSTRACT:Kaposi's sarcoma appeared on a chronically lymphedematous leg oi a 75‐year‐old man. lmrnunologic investigations revealed a lack of cellular immune response confined to the involved lower limb. Regional disorders of immune surveillance are thought to play an important role in the early stages of Kaposi's sarc

ISSN:0011-9059    

DOI:10.1111/j.1365-4362.1984.tb05664.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

ABSTRACT:Cutaneous fibrinolytic activity was found to be increased in a patient with aquagenic pruritus both before and after contact with water. This could explain the lack of wheal formation in this affection, which is characterized by increased histamine release.

ISSN:0011-9059    

DOI:10.1111/j.1365-4362.1984.tb05665.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY