Epidermolysis Bullosa Simplex is characterized by autosomal dominant rnheritance, and chronic noninflammatory conditions in which the clinical lesions, erosion, blisters usually result from relatively minor mechanical trauma to the skin, especially joints of hands, elbows, knees, and feet and other sites subject to repeated trauma The lesiona are present or appeared after a few days after birth or shortly after especialy of the lower legs or feet, and not involved at naiIs or mucous membranes. Though infection is not common on the feet or hands, even infected lesions generally heal with out scarring. 2 cases of Epidermolysis bullosa simplex were experienced, the one was 5 month old male baby who had fresh vesiculobullous, hemorrhagic bulla, crusts, exfoliation, no nail dystrophy, since a few days after birth. The another was 5 month old female baby, who had pea sized tense bulla, and exfoliations on the hands and feet. Diagnosis confirmed by clinical features and histological findings. Literature were reviewed in comparison with mechanobulIous disease.
Blister ; Cicatrix ; Diagnosis ; Elbow ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Female ; Foot ; Hand ; Humans ; Infant ; Joints ; Knee ; Leg ; Male ; Mucous Membrane ; Parturition ; Peas ; Skin ; Transcutaneous Electric Nerve Stimulation

Familial benign chronic pemphigus is characterized by a recurrent eruption of plaques of closely grouped vesicles that most frequently occurs about the neck, axilla and groin, singly or in combination with similar lesions in the intertriginous area. A 27-year-old male has had recurrent vesicles, fissures, maceration and crust formation in inguinal and perianal area for 3 yeare. The lesion manifested circinated form of vesicles, fissured and scaly patches over the inguinal, scrotal and perianal area. There were actively inflammatory border, resembling tinea cruris in the inguinal area. Authors diagnosed with clinical symptoms, laboratory examinations, and light and electron microscopic examination.
Adult ; Axilla ; Groin ; Humans ; Male ; Neck ; Pemphigus, Benign Familial* ; Tinea

No abstract available.
Adult* ; Humans

No abstract available.
Adult* ; Humans

The pathogenetic mechanism of renal dysfunction in renal amyloidosis is poorly understood. To evaluate the morphologic parameters which are correlated with renal function in this disorder, we have examined renal biopsies from 14 patients with renal amyloidosis by morphometry. Of the 14 patients, 8 were male and 6 were female. They were between 41 and 70 years of age. The serum concentration of albumin and creatinine were 2.1+/-0.7 mg/dl and 1.1+/-0.5 mg/dl, respectively. The 24-hour excretion of urinary protein was 7.9+/-5.2 g. Creatinine clearance was 62+/-23 ml/min/1.73m2. The mean glomerular volume (MGV) was (2.2+/-1.3) 10(6) micrometer3. The surface density of peripheral glomerular basement membrane [Sv (PGBM/glom)] was 0.049+/-0.027 (micrometer3/micrometer3). Volume density of mesangium [Vv (mes/glom)] was 0.31+/-0.14 (micrometer3/micrometer3) and volume density of glomerular amyloid deposition [Vv (amyl/glom)] was 0.21+/-0.14 (micrometer3/micrometer3). The volume density of cortical interstitium [Vv (int/cortex)] was 0.14+/-0.09 (micrometer3/micrometer3). The serum creatinine concentration was significantly correlated with Vv (int/cortex) (r=+0.66, p<0.05). MGV was correlated with Vv (mes/glom) (r=+0.75, p<0.01) and Vv (amyl/glom) (r= +0.68, p<0.05) but showed negative correlation with Sv (PGBM/glom) (r=-0.79, p<0.01). Sv (PGBM/glom) showed negative correlation with Vv (mes/glom) (r=-0.77, p<0.01) and with Vv (amyl/glom) (r=-0.87, p<0.01). Positive correlation was observed between Vv (mes/glom) and Vv (amyl/glom) (r=+0.95, p<0.01). These results suggest that the decreased renal function in patients with amyloidosis is related to interstitial fibrosis rather than glomerular lesions. In addition, glomerular hypertrophy in these patients is related to amyloid deposition in the mesangium and peripheral glomerular basement membrane.
Amyloidosis* ; Biopsy ; Creatinine ; Female ; Fibrosis ; Glomerular Basement Membrane ; Humans ; Hypertrophy ; Male ; Plaque, Amyloid

BACKGROUND: It is a routine practice that High Frequency Jet Ventilation (HFJV) is applied through a 14~16 gauge (about 12 French (Fr.)) angiocath. The 14 Fr. suction catheter which is similar to angiocath in its internal diameter is commonly available in the operating room. We evaluated the suction catheter as a carrier of HFJV in point of ventilation, oxygenation and operating field during HFJV at microlaryngeal endoscopic surgery (MES). METHODS: Fifteen adult patients undergoing HFJV via 14 Fr. suction catheter during MES were studied. Time-based arterial blood gas analyses were done before and after HFJV. The movement of operating field was examined using laryngoscopic examination by surgeon and anesthesiologist. We also evaluated complications such as abdominal distension, barotrauma and so on. RESULTS: The mean arterial oxygen tension was maintained above 250 mmHg all the time during HFJV. The mean carbon dioxide tension was less than 51 mmHg. There were no remarkable catheter movement and complications. CONCLUSION: The 14 Fr. suction catheter is a good replacement of angiocath. It provided good operating field, ventilation and oxygenation without complications.
Adult ; Barotrauma ; Blood Gas Analysis ; Carbon Dioxide ; Catheters* ; High-Frequency Jet Ventilation* ; Humans ; Operating Rooms ; Oxygen ; Suction* ; Ventilation

BACKGROUND: This study was conducted to compare the clinical and radiological outcomes of the locking hook plate fixation (HP) technique and the single tight rope (TR) technique applied for acute high-grade acromioclavicular (AC) joint separations. METHODS: Between 2009 and 2014, 135 consecutive patients with acute AC joint separation Rockwood types III, IV, and V were subjected to surgical reconstruction. One hundred fourteen patients (84.4%) were available for retrospective evaluation. Of them, 62 and 52 were treated using the single TR group and clavicular HP group techniques, respectively. The visual analogue scale, Constant, American Shoulder and Elbow Surgeons (ASES), and Taft scores were used for clinical assessment. Postoperative shoulder range of motion was also assessed. An anteroposterior radiograph of the coracoclavicular distance (CCD) was obtained to evaluate the radiographic signs of recurrence. RESULTS: The TR group patients had better Constant, ASES, and Taft scores than the HP group patients. The loss of reduction in terms of the CCD did not differ between groups. Subacromial osteolysis was observed in 34.6% of the cases in the HP group. However, there were no significant differences in the clinical outcomes between the patients with and without osteolysis in the HP group. Subcoracoid osteolysis, drill tunnel widening, and metal displacement were observed in 3.2%, 22.6%, and 4.8% of the cases in the TR group, respectively. CONCLUSIONS: The single TR technique was relatively more effective at treating acute high-grade AC joint injuries than the HP fixation technique (level of evidence: therapeutic; retrospective comparative study, Level III).
Acromioclavicular Joint* ; Dislocations* ; Elbow ; Humans ; Joints ; Osteolysis ; Range of Motion, Articular ; Recurrence ; Retrospective Studies ; Shoulder ; Surgeons

No abstract available.
Diagnosis* ; Facial Asymmetry*

No abstract available.
Diagnosis* ; Facial Asymmetry*

No abstract available.
Rectal Fistula*