ISSN:0385-2407    

DOI:10.1111/j.1346-8138.1991.tb03031.x

年代:1991

数据来源: WILEY

ISSN:0385-2407    

DOI:10.1111/j.1346-8138.1991.tb03032.x

年代:1991

数据来源: WILEY

摘要:

AbstractHLA in systemic scleroderma (PSS), including three familial cases, is reported. Three families in which one sister developed PSS and another sister suffered from either PSS (family 1), mixed connective tissue disease (MCTD) (family 2), or Sjögren's syndrome (SjS) (family 3) were described. The elder sister in family 1 died of respiratory insufficiency caused by scleroderma lung. The sisters in family 2 both had SjS, anti SS‐A antibodies, and HLA A2‐Bw55‐Cwl‐DRw8 haplotype in common. The elder sister with PSS in family 3 also had SjS and Hashimoto's thyroiditis. HLA in 28 PSS patients including these 3 familial cases were analyzed with 4 MCTD and 4 generalized morphea patients. HLA A2, Bw46, DR2, DRw8, DRw6 and DQw1 antigens were more frequently found in the PSS patients than in the controls. HLA DRw6 was the only antigen that was positive in common in the 3 familial cases. In those patients with anti topoisomerase I antibodies, HLA DR2 antigen was found more frequently than in the controls. Some, but not all, of these results were similar to the previous reports on HLA in PSS. Further investigations on more patients and the other members of these families would be necessary to clarify the significance of these

ISSN:0385-2407    

DOI:10.1111/j.1346-8138.1991.tb03033.x

年代:1991

数据来源: WILEY

摘要:

AbstractLabial lip biopsy findings from 52 patients with Sjögren syndrome (SjS) and cutaneous manifestations were clinically analyzed.Findings of the labial lip biopsy with more than one focus (a cluster of at least 50 lymphocytes) were positive in 52% (28/52). Sixteen patients who presented with oral dryness had negative labial lip biopsy findings with less than one focus score and five patients without oral dryness had positive results. Cells infiltrating into the salivary gland were categorized as plasma cell rich (Group 1), lymphocyte rich (Group 3), intermediate (Group 2), or no gland in biopsied specimens (Group 4). Sicca complaint and the grade of lip biopsy score were significantly higher in Group 3 than in Group 1; no differences were observed in laboratory findings between Groups 1 and 3. The patterns of age distribution and salivary flow rate were also not statistically significant in these two groups. The patients with plasma cell rich infiltrate presented oral dryness, although their clinical and grade of lip biopsy scores were relatively low; most of these patients have secondary SjS. Some of the patients in Group 4 showed an advanced stage of the disease though their lip biopsy score was grade 1 with atrophic or no gland. These data suggest that labial lip biopsy is a useful screening method for the diagnosis of Sjögren syndrome and that clinical manifestations may differ in parallel with the type of infiltrating cel

ISSN:0385-2407    

DOI:10.1111/j.1346-8138.1991.tb03034.x

年代:1991

数据来源: WILEY

摘要:

AbstractA case of xeroderma pigmentosum group D in a 36‐year‐old woman (XP85TO) is reported. The patient had severe photosensitivity from age 4, and developed multiple basal cell epitheliomas and solar keratoses but exhibited no apparent neurological defects. A skin phototest by monochromatic ultraviolet light revealed a delayed peak of erythema 48 h after irradiation and lowered minimal erythemal doses. Unscheduled DNA synthesis induced in XP85TO cells was 36.0% in dermal fibroblasts and 32.6% in epidermal keratinocytes compared with normal cells. The XP85TO cells were sensitive to ultraviolet killing (n=1.0, D0=0.80J/m2). In complementation analysis, XP85TO cells did not complement with xeroderma pigmentosum group D cells. These results indicate that patient XP85TO had xeroderma pigmentosum group D.The Japanese group D patients including XP85TO case showed delayed onset of skin malignant tumors and neurological abnormalities, compared with the group D patients in Europe and the United States. These findings suggest a possible ethnic variation of the clinical phenotype, despite the similar repair defect and ultraviolet hypersensitiv

ISSN:0385-2407    

DOI:10.1111/j.1346-8138.1991.tb03035.x

年代:1991

数据来源: WILEY

摘要:

AbstractThe average superoxide dismutase (SOD) activity of seven cell strains from mouse skin fibrosarcoma induced by ultraviolet (UV) irradiation was 5.78 ± 0.81 (mean ± SE) unit/mg protein, while that of seven normal mouse skin fibroblasts was 5.36 ± 1.58. Although tumor cells have been previously reported to exhibit reduced SOD activity, the present study demonstrates that there is comparable SOD activity between tumor cells and normal cells of the same tissue orig

ISSN:0385-2407    

DOI:10.1111/j.1346-8138.1991.tb03036.x

年代:1991

数据来源: WILEY

摘要:

AbstractAbnormal porphyrin metabolism can be induced by several chemicals. To investigate the synergistic effect on porphyrinopathy of isonicotinic acid hydrazide (INH) with a low concentration of griseofulvin (GF), the two chemicals were given to mice simultaneously. INH was added to drinking water at a concentration of 0.05%. GF was mixed with feed at a concentration of 0.1%. The mice (dd‐y strain) were divided into four groups. Those in group A were fed normally. Group B received only 0.1% GF, group C received only 0.05% INH, and group D received both 0.1% GF and 0.05% INH. The treatment was continued for 13 to 30 weeks. After treatment, the mice were anesthetized and sacrificed. The liver and whole blood were taken for analysis of porphyrins. The results revealed a slight elevation of erythrocytic porphyrins in the groups treated with 0.1% GF or 0.05% INH alone and remarkable abnormalities in the hepatic and erythrocytic porphyrin levels of the group simultaneously treated with both chemicals. These results show that INH itself may have a small potential for the induction of porphyric abnormalities, and that the administration of INH with a low concentration of GF induces marked porphyrinopathy in dd‐y strain m

ISSN:0385-2407    

DOI:10.1111/j.1346-8138.1991.tb03037.x

年代:1991

数据来源: WILEY

摘要:

AbstractA 38‐year‐old Japanese man was seen for severe palmoplantar keratoderma, extending to the dorsal aspects with red rims. He had had spontaneous amputations of the toes and surgical amputation of the right lower leg because of squamous cell carcinoma of the right sole. The clinical symptoms suggested those of mal de Meleda, except for the absence of consanguinity and of granular layers in the epidermis. The keratoderma improved with oral etretinate treatm

ISSN:0385-2407    

DOI:10.1111/j.1346-8138.1991.tb03038.x

年代:1991

数据来源: WILEY

摘要:

AbstractThree cases of striae distensae which developed along suture scars after skin surgery were described. The production of striae should be taken into consideration in surgery of the skin, especially in young woman.

ISSN:0385-2407    

DOI:10.1111/j.1346-8138.1991.tb03039.x

年代:1991

数据来源: WILEY

摘要:

AbstractA 27‐year‐old Japanese male student with tinea of the glans penis was described. The lesion on the glans penis was unique; it developed as a crop of papu

ISSN:0385-2407    

DOI:10.1111/j.1346-8138.1991.tb03040.x

年代:1991

数据来源: WILEY