The pathologic and immunopathologic findings of 15 skin biopsies of Behcet's syndrome were studied to investigate the pathogenetic mechanism of this syndrome. The age range was 28 to 50 years; male to female ratio was 1 : 1.2. Ulceration with acute necrotizing and chronic nonspecific inflammation was present in most cases with leukocytoclastic vasculitis and perivascular lymphohistiocytic, plasmocytic infiltrations. Eight of 15 cases showed C3 deposit in the walls of venules and capillaries, one of them was associated with IgM and fibrinogen deposits. With these findings humoral factor seemed to play a main role in the pathogenesis of vasculitis observed in Behcet's syndrome, but the possibility of other mechanisms operative in complex pattern was also considered.
Female ; Male ; Humans ; Biopsy

Articular cartilage can be damaged by trauma or by progressive degradation caused by disease. Unfortunately, it has a extremely limited capacity for repair and regeneration. The use of joint prosthesis has revolutionized but remains limited in its use. To know the possibility of clinical application of clinical application of autogenous bone-articular graft in open complicated intraarticular fractures we studied the change of bone-articular cartilage defect in medial femoral condyle of rabbits and viability of autogenous bone-articular graft stored within abdominal wall of rabbits. The results were obtained as follows: 1. There was over 90% viability of subchondral bone after 10 days storage in the adbominal wall but the articular cartilage showed degenerative change about 40% after 5 days passed. 2. The bone-articular defect was replaced by undifferentiated mesenchymal tissue and fibrous tissue, postoperative 6 weeks later. 3. In implanted bone-articular cartilage graft at 3 days, the chondrocyte of basal cell layer in articular cartilage was relatively well preserved but in 7 days, transplanted articular cartilage graft showed almost necrotic chage. Most of subchondral bones were well healed with host bone without regard to duration of implantation.
Abdominal Wall ; Cartilage ; Cartilage, Articular ; Chondrocytes ; Intra-Articular Fractures ; Joint Prosthesis ; Rabbits ; Regeneration ; Transplants

Hyperkeratosis of the renal pelvis and ureter is a rare condition and is explained by the metaplastic change from transitional to cornified squamous epithelium. This lesion is frequently associated with, and perhaps caused by chronic urinary infection, but potentially premalignant, accompaning with carcinoma in about 10 to 20 per cent of the cases at the time of diagnosis. A case of hyperkeratosis of renal pelvis & ureter which was clinically suspected of carcinoma in a 49-year old woman with a long history of chronic pyelonephritis is presented with review of literature.
Female ; Humans

To know the correlation between glomerular and tubulointerstitial lesion and to define the characteristics of interstitial inflammatory cell in IgA nephropathy and classified according to WHO classification and graded tubulointerstitial lesion as mild, moderate and severe. Paraffin-embedded 5u sections were stained with UCHL-l, L26 and CD68 antibodies. More than 20 fields were examined in each case under the high power microscopy and the number of positive cells were counted. There was positive correlation between the severity of glomerular and that of tubulointerstitial lesion. The mostcommoninflammatory cells in the interstitiuin were UCHL-l positive cells followed by CD68 and L26 positive cells. As the WHO grade or tubulointerstitial lesion increased, the numbers of positive cells were increased in all three groups. The proportion of UCHL-1 Positive cells were increased in cases with high WHO grade whereas that of L26 positive cells incases with severe tubulointerstitial lesion Proteinuria was correlated with the degree of inflammatory cell infiltration, especially with that of L26 positive cells.

The rapid frozen section method is a means of intraoperative pathological diagnosis, first introduced by Welch in 1891 and developed as a diagnostic tool by Cullen, Wilson, MacCarty et al. This method serves useful purposes, such as determining the malignancy or benignancy of a suspected lesion, determining the adequacy of a biopsy of a suspected lesion, confirming the presence or absence of metatasis, and identifying small structures. But it bears many disadvantages, the most of which is the danger of incorrect diagnosis. We studied the indications, the limitations and the accuracy of the frozen section method and the materials studied was total cases of frozen section during recent 5 years. The ovarall accuracy of the frozen section diagnosis of 1,603 cases was 96.2% with 0.3% of false positive, 3.5% of false negative and 2.8% of incorrect histological diagnoses or grading errors the tissues submitted for frozen section were lymph node, breast, gastrointestinal tract and soft tissue in decreasing order of frequency. The false positive cases were four in number, while the false negative cases were 53, one third of which were the misdiagnoses of the presence of ganglion cells in Hirschsprung's disease.
Biopsy

Soft tissue tumor is defined as a tumor occurring in voluntary muscles, fat, fibrous tissue, along with the vessels serving these tissue and peripheral nervous system. It is difficult to make a diagnosis by conventional microscopic observation because of their pleuripotentiality and similar growth characteristics. Although their morphological findings of tumors are similar to one another, their clinical courses, treatment and prognosis are different. So early, correct diagnosis and proper treatment are neccessary. The present study is aimed to evaluate a value of immunoperoxidase staining to make definite diagnosis of soft tissue tumors and its application to surgical pathology. The material consisted of 106 cases of fibrohistiocytic tumors and malignant soft tissue tumors which are morphologically similar to malignant fibrohistiocytic tumors for 5 years period lasting from 1980 to 1984 at the Department of Pathology, Yonsei University College of Medicine. After the classificationof fibrohistiocytic tumors by the Enzinger (1983), clinical finndings were reviewed and peroxidase antiperoxidase(PAP) method with alpha1-antichymotrypsin was done in 15 cases of all fibrohistiocytic tumors. Other soft tissue tumors which were difficult to differentiate from MFH by light microscopic observation were liposarcoma, rhabdomyosarcoma, fibrosarcoma and malignant schwannoma. These 21 cases of tumors including MFH were stained with PAP method for alpha1-antichymotrypsin, S-100 protein and myoglobin. Results obtained were as follows: 1) The cases on study consisted of 19 cases of malignant fibrous histiocytoma, 2 dermatofibrosarcoma protuberans, 45 fibrohistiocytic tumors and 11 other benign fibrohistiocytic tumors. 2) The male to female ratio was 1 : 1.8 in benign and intermediate group of fibrohistiocytic tumor, but 2.2 : 1 in malignant histiocytic tumor. 3) Most cases of benign fibrohistiocytic tumors were occurred in 4th and 5th decade of life. Intermediate and malignant fibrohistiocytic tumors were mostly found in late adult life and their mean age was 43.6 year. 4) The most common sites were trunk and both extrimities in benign fibrohistiocytic tumors(88.9%), but head, neck and lower extremities in MFH (78.9%). Two cases of dermatofibrosarcoma protuberans were occurred in turnk and upper extremity. 5) The PAP stain for alpha1-antichymotrypsin was done in 15 cases of 77 fibrohistiocytic tumors which included MFH, dermatofibrosarcoma protuberans, xanthoma, xanthofibroma, dermatofibroma showed variable degree of positivity to alpha1-antichymotrypsin. The positivity of alpha1-antichymotrypsin revealed no significant difference according to differentiation of the tumors, such as benign, intermediate and malignant. 6) The PAP stain for alpha1-antichymotrypsin revealed diffuse positivity in all cases of MFH and also in a case of malignant schwannoma, fibrosarcoma, liposarcoma and rhabdomyosarcoma, but myoglobin and S-100 protein were negative. In three cases of leiomyosarcoma, two of rhabdomyosarcoma and three of malignant schwannoma, alpha1-antichymotrypsin, S-100 protein and myoglobin were negative, although a few positive tumor cells were present, which may the considered as metatypci differentiation. Another possibility of this discordance was loss of antigenicity by improper procedure of paraffin embedding and poor differentiation of tumor cells. In summary, PAP method for specific tumor marker is important for proper diagnosis of soft tissue tumors, and application to surgical pathology.
Adult ; Male ; Female ; Humans ; Tumor Markers, Biological

No abstract available.
Nephrotic Syndrome*

Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by concurrent nevus flammeus (capillary malformation) and pigmentary nevus. According to current research, the major pathophysiologic mechanism in PPV is venous dysplasia with resultant compensatory collateral channels and venous hypertension. Arterial involvement is rare. We herein report our experience on renovascular hypertension, intermittent claudication, and severe rhabdomyolysis due to diffuse stenosis of multiple arteries in a patient with PPV type IIb associated with SWS.
Arteries ; Constriction, Pathologic ; Humans ; Hypertension ; Hypertension, Renovascular ; Intermittent Claudication ; Intracranial Aneurysm ; Neurocutaneous Syndromes* ; Nevus ; Port-Wine Stain ; Rhabdomyolysis* ; Sturge-Weber Syndrome* ; Vascular Diseases

Twenty-five cases of minimal change nephrotic syndrome(minimal change disease, MCD) with mesangial IgA deposition were evaluated electron microscopically. The thickness of the glomerular basement membrane(GBM) was 3875 +/- 1271 A and 3056 +/- 1201 A in adults and children, respectively. Alteration of the GBM was noted in 3 adults and eight children: splitting in 4, focal thinning in one, widening of the lamina rara interna in 10, and widening of the lamina rara externa in 4 cases. Minimal mesangial electron dense deposits were found in all but one adult, and an increase of the mesangial matrix and minimal mesangial proliferation were observed in 8 and 6 cases, respectively. Electron microscopic findings show representative findings of MCD in our cases. A relationship between the GBM alterations in these cases and frequent association of hematuria is suggested and discussed.
Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Glomerular Mesangium/*metabolism ; Human ; Immunoglobulin A/*metabolism

No abstract available.
Nephritis, Hereditary*