Generalized pustular psorissis which is originary described by von Zumbusch in 1910, is the most intense form of the acute exanthematic variant among psoriasis and it is to be occured as a rare disease. We presented a case of generalized pustular psoriasis of a 16 year-old girl, who suddenly developed wide spread pustules during the acute exacerbating course of the psoriasis which had started from the primary plaque on her left anterior chest one month ago. She was free from subjective symptom. As the objective sign, the leukocytosis was only one of the significant laboratory findings. All skin lesions were almost eleared by the oral administration of methotrexate in 3 months.
Administration, Oral ; Adolescent ; Female ; Humans ; Leukocytosis ; Methotrexate ; Psoriasis* ; Rare Diseases ; Skin ; Thorax

We report a case of epidermolysis bullosa acquisita in a 53 year-old woman who had. extreme skin fragility, trauma induced blisters and erosions usually localized to extensor site of the skin surface, and healing with scars and milia for several years. Histologic findings of perilesional skin showed normal epidermis, subepid malblister and spirsely infiltration of inflammatory cells. Direct immuncofluorescence showed depositiori of IgCi and C3 in a linear pattern along the epidermal basement, membrane in the per ilesional skin. In indirect immuriofluorescence, using 1.0M sodium chloride separated nor mal human skin as the sntbstrate, antitodies(IgG at a titer of 1:40) were bound to dermal site of the separation, indicating that the patient serum yields the reaction in or below the lamiria densa. Electronmicroscopic findings showed the roof of the blister was located. below the basal lamiria.
Blister ; Cicatrix ; Epidermis ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Female ; Humans ; Membranes ; Middle Aged ; Skin ; Sodium Chloride

PURPOSE: To evaluate the findings and the role of CT in plexiform neuro-fibromatosis of the mediastinum. MATERIALS AND METHODS: We retropectively reviewed the CT scans of five patients with plexiform neurofibromatosis of the mediastinum. The CT scans were reviewed with attention to the distribution of the lesions, appearance and attenuation of mediastinal lesions, enhancement pattern after intravenous contrast infusion and associated findingssuch as intercostal neurofibroma. RESULTS: In all five patients CT scans demonstrated fusiform low attenuated masses which were oriented longitudinally and extended over multiple contiguous scans along the distribution of major mediastinal nerves. In four patients, mediastinal lesions appeared infiltrative, obliterating adjacent mediastinal fat plane. One patient had well defined fusiform masses along the major mediastinal nerves. Postcontrast enhanced CT scans revealed slight central enhancement in two patient and no contrast enhancement in three patients. Associated findings such as neurofibromas of intercostal nerves and sympathetic trunk, or subcutaneous neurofibromas were detected on CTscans in all five patients. CONCLUSION: Characteristic CT findings of low attenuation masses along the major mediastinal nerves are helpful to differentiate plexiform neurofibromatosis from mediastinal lymphadenopathy and to prevent from misreading as a malignant disease.
Humans ; Intercostal Nerves ; Lymphatic Diseases ; Mediastinum* ; Neurofibroma ; Neurofibromatoses* ; Tomography, X-Ray Computed

The authors performed the interfascicular nerve grafting in the gap of injured nerves using the microsurgical technique to obtain the accurate coaptation of the fascicles. The authors experienced the 33 cases(26 patients) of the interfascicular nerve grafting in the median(11), ulnar(9), radial(2), common peroneal(3) and digital(8) nerves at the Department of Orthopaedic Surgery, College of Medicine, Kyung Hee University from Jan. 1980 to June 1984. The patients were followed up from 12 to 26 months (average 20 1/2 months). The following results were obtained; 1. The shorter the length of the donor nerve and the younger the age of the patients are, the better the recovery of the nerve function is. 2. The medial and lateral cutaneous nerves of forearm as donor nerve are suitable as well as the sural and superficial radial nerves. 3. The end result of each patient was evaluated by Seddon's classification. The excellent motor recovery was 72.0% (18/25) and the excellent sensory recovery was 81.8% (27/33). 4. The interfascicular nerve grafting is an excellent method in treating the widely separated,severed peripheral nerve injury.
Classification ; Forearm ; Humans ; Methods ; Peripheral Nerve Injuries ; Peripheral Nerves ; Radial Nerve ; Tissue Donors ; Transplants

Primary biliary cirrhosis(PBC) is a chronic cholestatic liver disease of unknown origin. The small and medium sized intrahepatic bile ducts are destroyed by an inflammatory process, which, it has been suggested, is of the autoimmune type. It is strongly associated with the presence of antimitochondrial antibodies, predominantly IgM and IgG. The liver changes are classified into four stages, of which stage IV represents the development of cirrhosis, which required orthotropic liver transplantation in the longrun. The prevalence rates was reported 128 per millon in Sweden , but the disease is relatively rare in Oriental area. In medical treatment, long-term administration of ursodeoxycholic acid improves both clinical and biochemical signs, slows the progression of the disease and reduces the complication requiring liver transplantation. We report two cases of PBC, one with histologically proven cirrhosis, and the other with bile duct destruction consistent with stage III and hypothyroidism.
Antibodies ; Bile Ducts ; Bile Ducts, Intrahepatic ; Fibrosis ; Hypothyroidism ; Immunoglobulin G ; Immunoglobulin M ; Liver ; Liver Cirrhosis, Biliary* ; Liver Diseases ; Liver Transplantation ; Prevalence ; Sweden ; Ursodeoxycholic Acid

BACKGROUNDS/AIMS: To investigate survival rates and prognostic factors of patients with gallbladder cancer who were treated with surgery and postoperative radiation therapy. METHODS: Seventeen gallbladder cancer patients who received surgery and postoperative radiotherapy from October 1989 to April 1998 were included in this retrospective study. Five patients had stage II, 8 patients had stage III, and 4 patients had stage IV disease according to the 1997 American Joint Committee on Cancer (AJCC) staging. All patients received > or =40 Gy of postoperative radiotherapy with a daily dose of 2.0 Gy/fraction and 15 patients received concurrent chemotherapy. An analysis was performed for the endpoints of overall and disease-free survival. RESULTS: Of the 17 patients, 13 had no residual disease (R0), 1 had microscopic residual disease (R1), and 3 had macroscopic residual disease (R2) after surgery. Among patients with no residual disease, 4 had locoregional recurrences during the follow-up period. One patient with microscopic residual disease had local recurrence. The 5-year overall survival rate was 38.2%. The median overall survival time was 21 months and the median disease-free survival time was 12 months. Old age (> or =60 years old), female gender, a high pathological stage (> or =IVA), and the presence of residual disease after surgery were significant prognostic factors for disease-free survival. CONCLUSIONS: Despite a high proportion of patients with advanced disease and macroscopic residual disease, the prognosis of gallbladder patients who had postoperative radiotherapy is encouraging. Additional investigation to improve the loco-regional control of gallbladder cancer patients with adverse prognostic factors is warranted.
Disease-Free Survival ; Female ; Follow-Up Studies ; Gallbladder ; Gallbladder Neoplasms ; Humans ; Joints ; Prognosis ; Recurrence ; Retrospective Studies ; Survival Rate

We experienced five cases of solitary fibrous tumor; two in the pleura, two in the orbital soft tissue, and one in the lung parenchyma. Three patients were male, and the age of the patients ranged from 38 to 71 years (mean age: 53.6). Grossly, the masses were well circumscribed and had varying sizes from 2.5 to 30.0 cm. The cut surfaces were grayish-yellow firm with focal variegated hemorrhage, necrosis, cystic change, and myxoid area. Microscopically, these were characterized by a haphazard proliferation of spindle cells or polygonal cells separated by variable amounts of hyalinized collagen and showed a prominent vascular channels reminiscent of hemangiopericytoma in foci. Immunoperoxidase stains showed a strong reactivity for CD34, and were weakly positive for vimentin. Electron microscopical examination revealed features of fibroblast; spindle to round tumor cells were arranged in groups and surrounded by collagen. Nucleoli were seldom prominent. The cytoplasm contained many microfilaments and a moderate number of cisternae of rough endoplasmic reticulum.
Actin Cytoskeleton ; Collagen ; Coloring Agents ; Cytoplasm ; Endoplasmic Reticulum, Rough ; Fibroblasts ; Hemangiopericytoma ; Hemorrhage ; Humans ; Hyalin ; Lung ; Male ; Necrosis ; Orbit ; Pleura ; Solitary Fibrous Tumors* ; Vimentin

Bailey and Cushing described the oligodendroglioma firstly in 1926. The oligodendroglioma is rare tumor and involves the ventricular system in 7% to 10% of all cases. The authors present a case of intraventricular oligodendroglioma associated with arterio venous malformations in a 34 year old male who had the history of visual disturbance and mental deterioration, for one month. The diagnosis was confirmed by operation and autopsy.
Adult ; Autopsy ; Diagnosis ; Humans ; Male ; Oligodendroglioma*

Thirty eight patients with stage Iand II primary gastrointestinal non-Hodgkin's lymphoma were treated in the Department of Therapeutic Radiology, Seoul National University Hospital between 1979 and 1984. There were 6 systemic disseminations during radiotherapy, and the overall failure rate were 31% in the cases with tumor bulk less than 5 cm in diameter before radiotherapy and 75% in the cases with tumor bulk greater than 5 cm in diameter (p<0.05). The overall 5 year survival rate were 69.2% in 28 patients who completed radiotherapay and 72% in 24 patients with tumor bulk less than 5 cm in diameter (small or no tumor bulk). The 5 year disease free survival rate were 71% in cases with tumor bulk less than 5 cm in diameter and 25% in cases with tumor bulk greater than 5 cm in diameter (p<0.01). But the initial stage was not related with treatment result in all cases or subgroups of cases. Thus the cases with small or no tumor bulk were shown to be curable with combined surgery and postoperative radiotherapy, but for the control of the cases with large tumor bulk that had a guarded prognosis combined radiotherapy and chemotherapy should be tried.
Disease-Free Survival ; Drug Therapy ; Humans ; Lymphoma, Non-Hodgkin* ; Prognosis ; Radiation Oncology ; Radiotherapy* ; Seoul ; Survival Rate

PURPOSE: This study was undertaken to analyze the efficacy and sphincter preservation rate of platinum based neoadjuvant chemotherapy plus radiotherapy versus abdominoperineal resection and postoperative radiotherapy for anal cancer. MATERIALS AND METHODS: Data of forty-two patients with anal cancer were retrospectively analyzed. Among thirty-eight patients with epidermoid histology, four patients received radiotherapy, and nineteen patients received abdominoperineal resection and adjuvant radiotherapy with or without chemotherapy (APR+RT+/-CT), and fifteen patients received neoadjuvant chemotherapy and radiotherapy (CRT). The CRT regimen was composed of three cycles of 5-fluorouracil (1,000 mg/m2 bolus on D1-5) and cisplatin (60 mg/m2 bolus on D1) followed by 50.4 Gy to the tumor bed and regional lymphatics over 5.5 weeks. Both inguinal lymphatics were treated with an identical dose schedule. Residual disease was treated with an additional three cycles of identical adjuvant chemotherapy. An identical dose schedule was used for post-operative radiotherapy. Median follow-up period was eighty-five months. RESULTS: Overall five-year survival rates were 80.3%, 88.9 and 79.4% for entire patients, APR+RT+/-CT group, and the CRT group, respectively. No significant difference was found between the two groups (p= 0.49). Anus preservation rate for the CRT group was 86.7%. Age (p=0.0164) and performance status (p= 0.0007) were found to be significant prognostic factors by univariate analysis. Age (p=0.0426), performance status (p=0.0068), and inguinal lymph node metastasis (p=0.0093) were statistically significant prognostic factors by multivariate analysis. No case of RTOG grade 3 complication or higher was reported. CONCLUSION: This and other recent studies have shown that combined chemotherapy plus radiotherapy for anal cancer results in a high rate of anal sphincter preservation as well as local control and survival. Furthermore, neoadjuvant use of chemotherapy with a cisplatin based regimen rather than a concurrent regimen may lead to a decrease in complications.
Anal Canal ; Anus Neoplasms* ; Appointments and Schedules ; Chemoradiotherapy ; Chemotherapy, Adjuvant ; Cisplatin ; Drug Therapy ; Fluorouracil ; Follow-Up Studies ; Humans ; Lymph Nodes ; Multivariate Analysis ; Neoplasm Metastasis ; Platinum ; Radiotherapy ; Radiotherapy, Adjuvant ; Retrospective Studies ; Survival Rate