Epidermolysis bullosa simplex (EBS) is a group of autosomal dominantly inherited genetic disorders characterized by blistering due to mechanical- stress-induced degeneration of basal epiderrnal cells. Recently, it was discovered that EBS is induced by keratin 5 and 14 gene mutations. Weber Cockayne (W-C) EBS is the mildest type, with blistering concentrates primarily on palar and plantar regions, and basal cell cytolysis by keratin filament perturbations is present. Herein we report a case of W-C EBS with its ultrastructural findings. Electron microscopy showed cytolysis and separation of the basal epidermal cells, mainly at the subnuclear cytoplasm. The cyto- plasm of basal cells showed edema, loosening and intact rnitochondria. Besides the cytoplasmic changes, the nucleus also showed lytic degeneration. Characteristically, dense condensation of tonofilarnent was observed, which suggests that W-C EBS is. also a disorder of keratin.
Blister ; Cytoplasm ; Edema ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Keratin-5 ; Microscopy, Electron

No abstract available.
Continuous Positive Airway Pressure* ; Ventilation*

We reviewed five cases of Uterine leiomyomas, each with a conspicuously, yellow cut surface, among 198 consecutive cases of surgically removed uterine leiomyomas. Their gross findings were not significantly different from ordinary leiomyomas except for their pale to bright Yellowish cut surface. Microscopically, multiple small clusters of clear cells were widely scattered in otherwise hypercellular leiomyornas in 4 of the 5 cases. Of those, one case gave a positive reaction of Oil-Red O stain. Ultrastructurally, clear cells corresponded to the degenerating smooth muscle cells with intracytoplasmic lipid vacuoles. The rest of cells showed myofibers undergoing varying degrees of degeneration. Focal accumulation of foamy histiocytes was associated with carneous degeneration in one case. We conclude that the yellowish leiomyoma of the uterus seems, in part, to reflect accumulation of a lipid substance in degenerating hypercellular leiornyoma, or possibly collections of xanthoma cells in secondary degeneration.

No abstract available.
Pneumonia*

The aim in arthrodesis of ankle is to gain a painless, normal walking gait of destroyed ankle joint caused by various origins. Despite more than 30 different fusion techniques have been described in the literature, but the results were not always satisfactory. Blair introduced tibial sliding graft, rectangular hone block donated at distal tibia, to the neck of talus. His method has advantages of a normal apperance of the foot, no shortening, and the weight bearing thrust on normal tissue. The purpose of this study was to compare the ankle arthrodesis according to surgical procedures & introduce the method which could produce excellent results for ankle arthrodesis. We reviewed 23 patients(24 cases) undergone ankle arthrodesis from Jan. 1989 to Dec. 1996 in Kyung Hee Medical Center. Male were twelve(13 cases) and female were eleven. Mean age was 42.6(l8-75)years. The Blair fusion method was used in ten cases. Iliac bone graft followed by internal fixation using Steinmann pin in 4 cases, using cancellous screw in I case, and using both Steinmann pin and staple in 1 case, while tibiotalar coaptation without bone graft followed by internal fixation using Steinmann pin in 3 cases, using staple in 1 case, and using cancellous screw in 1 case. In 3 cases, arthroscopic abrasion and cancellous screw was used. The mean duration of follow-up was 46(6-99) inonths. Time to union was average l4.6(8-23) weeks in Blair fusion and 17.5(8-40) weeks in other methods. Delayed union was observed in three cases, but no case was found in Blair fusion. Pain was ohserved in 5 cases, 3 in Blair fusion and 2 in other methods. Nonunion or pseudoarthrosis was not ohserved. On the clinical criteria for Boston Childrens Hospital ankle-scoring system, we ohserved nineteen excellent and five good cases. The difference in results according to fusion method was not ohserved. The results in tibiopedal motion using cineroentgenography were seven good and three fair cases in Blair, while two good, ten fair and two poor in other methods. By using Blair fusion, we expect early bony union and better clinical results.
Ankle Joint ; Ankle* ; Arthrodesis* ; Child ; Female ; Follow-Up Studies ; Foot ; Gait ; Humans ; Male ; Neck ; Pseudarthrosis ; Talus ; Tibia ; Transplants ; Walking ; Weight-Bearing

The increased incidence of the trochanteric fracture of the femur was accompanied by the development of the vehicle and the prolongation of the average life-span. Recently it has been also increased in an active person from thirties to forties. 87 cases of the trochanteric fracture of the femur in 86 patients who have been admitted and treated at the Department of Orthopedic Surgery of Hanyang University Hospital during 8 years and 10 months, from May, 1972 to February, 1981, were reviewed. The results of this study were as follows: 1. The trochanteric fracture of the femur was more common in male and was frequent in a person who was from thirties to forties and who had vigorous social activity. 2. An injury from traffic accident was the most common cause of the fracture from thirties to forties and an injury from slip down was most common in seventies. 3. The unstable fracture which was classified by Evans' classification, Boyd and Griffin Type II Fx. and Tronzo Type III Fx. were most common in the intertrochanteric fracture and the Fielding Type II Fx. was most common in the subtrochanteric fracture of the femur. 4. Other injuries were accompanied by the trochanteric fracture of the femur in 38 patients (44.2%) and the pelvic bone fracture was the most common associated injury in these patients. 5. The weight bearing was allowed earlier in the patient who had been given the surgical Tx. than in the patient who had been given the conservative Tx. 6. In adults, there was no significant differences in the duration to achieve the bony union between the conservative treatment and surgical treatment performed. 7. We considered that the diminution of the probable complications by early weight bearing after accurate open reduction in accordance with the Type of the Fx. followed by secure internal fixation with devices is an ideal method of the Tx.
Accidents, Traffic ; Adult ; Classification ; Clinical Study ; Femur ; Hip Fractures ; Humans ; Incidence ; Male ; Methods ; Orthopedics ; Pelvic Bones ; Weight-Bearing

Hypereosinophilic syndrome is characterized by marked eosinohia and eosinopbilic infiltrition of various organ-systems without any identifiable underlyin sathologic conditions such as hypersensitivity, drug eruption, parasitic infestat,ion, malignant tuinc, and autoimrnune and infectious d iseases. A 31 year-old male had weight loss, abnormal peripheral cirrultiction both extremities and two distinct skin lesions. One showed rice sized, erythematous follicular popules on both extremit.ies and trunk and the other showed a walnut sized, gangrenous ulcer on the left 2nd finger tip. The patients skin lesions and circulation defect. improved after retrnent with steroid and the blood eosinophilia returned to normal.
Adult ; Blood Circulation* ; Drug Hypersensitivity ; Eosinophilia ; Extremities* ; Fingers ; Humans ; Hypereosinophilic Syndrome* ; Juglans ; Male ; Skin ; Ulcer ; Weight Loss

Pilomattiaoma, aften called calcifying epithelioma of Malherbe, is a benign tumor originating from the outer root sheath cell of the hair follicle and extending into the hair matrix, Pilomatricoma usually occurs as a single, asymptomatic dermal or subcutaneous nodule. Multiple lesions are quite unueuel, comprising only 2-3.5% of cases. We report two patients with multiple pilomatricoma.
Hair ; Hair Follicle ; Humans ; Pilomatrixoma*

No abstract available.
Fertilization in Vitro* ; Humans ; Twins* ; Umbilical Cord*

A 34-year-old woman presented with several intensely pruritic erythematous serpiginous thread-like skin lesions which began as a small papule on the epigastrium 4 months prior to her visit and was migrating to the left chest area. The laboratory examination showed eosinophilia and the total serum IgE level was slightly increased. Histopathologic examination revealed a pustular burrow in the epidermis with a larva-like cystic lesion apart from the burrow in the epidermis. The patient was treated with topical 10 % albendazole cream 3 times daily for 1 week without recurrence for 22 months up to now. We thought this was a rare case which was confirmed by a skin biopsy showing larva in the epidermis.
Adult ; Albendazole ; Biopsy ; Eosinophilia ; Epidermis ; Female ; Humans ; Immunoglobulin E ; Larva ; Larva Migrans* ; Recurrence ; Skin ; Thorax