Familial benign pemphigus(Hailey-Hailey disease) is a rare hereditary dermatosis that begins in the 2nd or 3rd decade of life. The skin lesion is characterized by a localized, recurrent eruption of small vesicles on an erythematous base. During its course there are remissions and exacerbations. It seldom begins in early childhood or after the age of 50. The main treatment modalities are conservative ones. A 59-year-old woman with familial benign chronic pemphigus presented with a 10 year history of generalized pruritic recurrent skin lesions on her neck, axilla, inguinal, antecubital, and trunk area. Physical examination showed moist, macerated, fissured and scaly patches on an erythematous base in the axillae, groins, neck, antecubital, and trunk. A biopsy specimen showed extensive suprabasal separation containing acantholytic cells.
Axilla ; Biopsy ; Female ; Groin ; Humans ; Middle Aged ; Neck ; Pemphigus* ; Pemphigus, Benign Familial ; Physical Examination ; Skin ; Skin Diseases

No abstract available.
Knee*

Diffuse neonatal hemangiomatosis is a fatal disorder characterired by multiple cutaneous and visceral hemangiomas. The organs most commonly affected are the gastrointestinal tract, brain, liver and lung. The complications are high output cardiac failure, gastrointestinal bleeding and hydrocephalus. We present a patient with diHuse neonatal hemangiomatosis associated with massive hepatic involvement and high output cardiac failure, which was successfully treated with high dose corticosteroid.
Brain ; Gastrointestinal Tract ; Heart Failure ; Hemangioma ; Hemorrhage ; Humans ; Hydrocephalus ; Liver ; Lung

Maffuccis syndrome was first reported by Maffucci, in 1881. This syndrome is manifested by multiple enchondromas and hemangiomas, characterized by non-hereditary mesenchymal neoplasia that has a propensity for malignant transformation. Involvement of long bones, particularly the bones of the hands and feet, is most common. The most important complication of this syndrome is the high frequency of chondr osarcomatous metaplasia. We report a case of Maffuccis syndrome in a 56-year-old woman with multiple hemangioma on both the hands and forearms, left foot and enchondromas of multiple bones. The disease started at the age of 8 and was associated with deformity, but malignant transformation was not found.
Chondroma ; Congenital Abnormalities ; Enchondromatosis ; Female ; Foot ; Forearm ; Hand ; Hemangioma ; Humans ; Metaplasia ; Middle Aged

BACKGROUND: As co-factors of metalloenzymes, zinc(Zn) and copper(Cu) have a considerable effect on nearly all the metabolism that takes place in organs of the body, including the skin. OBJECTIVE: The purpose of this study was to evaluate zinc and copper status in alopecia areata patients. METHODS: We measured serum levels of zinc and copper in 30 cases of alopecia areata patients and 10 normal individuals by atomic absorption spectrometry. Furthermore, we studied the significance of the ratio between serum levels of the two metals. RESULTS: The serum levels of zinc were significantly lower in alopecia areata patients than in healthy controls. The serum levels of copper were slightly lower in alopecia areata patients than in healthy controls. Ratios of Cu/Zn were slightly higher in alopecia areata patients than in healthy controls. But their differences were not statistically significant. CONCLUSION: We suggest that a serum Zn assay should be included in the chemical assessment of patients with alopecia areata.
Absorption ; Alopecia Areata* ; Alopecia* ; Copper* ; Humans ; Metabolism ; Metals ; Skin ; Spectrum Analysis ; Zinc*

Benign symmetric lipomatosis is characterised by diffuse symmetric deposits of adipose tissue, predominantly on the neck, shoulder, back and upper extremities. We report a case of benign symmetric lipomatosis in a 61-year-old-man, which was first noted one year previously. It began growing rapidly about 3 months ago and was associated with alcoholic liver disease. Histopathological findings showed that the reticular dermis had been replaced by normal uncapsulated mature fat cells that were slightly increased in number in the fibrous connective tissue. He was treated with theophylline 300mg/day for two months.
Adipocytes ; Adipose Tissue ; Connective Tissue ; Dermis ; Lipomatosis* ; Liver Diseases, Alcoholic ; Neck ; Shoulder ; Theophylline ; Upper Extremity

OBJECTIVE AND METHODS: To determine correlations among ambulatory renin activity, ambulatory blood pressure and microalbumin excretion rate, 66 Korean essential hypertensives were studied after 4 week wash-out period. The ambulatory blood pressure was monitored every 30 minutes and mean BP were calculated automatically. Urinary microalbumin excretion rate(UAER) and ambulatory plasma renin activity(aPRA) collected at mid-day were measured by radioimmunoassays. Subjectives were divided into 2 groups by aPRA value(2ng/ml/hr). RESULT: 14 cases were high renin group and 52 cases low renin group. The mean BP were 148.83/94.69mmHg in low renin group, and 146.57/98.07mmHg in high-renin group without difference. UAER were not different also between both groups. 23.07%(4/14) of non-dippers were included in high renin group and 25.58%(12/52) in low renin group without statistical difference. The aPRA was significantly related to UAER and systolic and diastolic mean blood pressure. Also UAER was related significantly to day mean blood pressures. CONCLUSION: Thus aPRA is thought to be a meaningful indicator to predict hypertensive renal target organ damage as well as blood pressure measured with 24-hr ABPM.
Blood Pressure* ; Hypertension* ; Plasma* ; Radioimmunoassay ; Renin*

Most renal carcinomas are thought to originate from the epithelial cells of proximal convoluted tubules in the renal cortex. Collecting duct carcinoma is a recently recognized histological variety of renal cell carcinoma considered to arise from the epithelium of the collecting ducts. It is important to distinguish the collecting duct carcinoma from ordinary renal cell carcinoma, because which has an aggressive clinical course with early metastasis and death. But in patients, the natural course of the disease and its response to treatment have not been clearly established. Herein we report a case of collecting duct carcinoma of kidney in 38-year-old woman who had generalized edema and left flank dull pain. The preoperative diagnosis was left renal cell carcinoma on physical examination and radiologic finding, and left radical nephrectomy was done.
Adult ; Carcinoma, Renal Cell* ; Diagnosis ; Edema ; Epithelial Cells ; Epithelium ; Female ; Humans ; Kidney* ; Neoplasm Metastasis ; Nephrectomy ; Physical Examination

In postanesthetic period, infants and children have a risk of hypoxemia due to decreased functional residual capacity(FRC) and increased alveolar-arterial O2 tension gradient(A-a DO2). We measured arterial oxygen saturation(SaO2) with a pulse oximeter in 60 ASA class 1 infants and children. Group 1 was breathing with supplemental oxygen(4L/min) by mask and Group 2 was breathing with room air in recovery room after general anesthesia. SaO2 was measured on arrival in the recovery room, 2,4,6, 8,10,15, and 20 minute after arrival The results were as follows: 1) Postanesthetic SaO2 measured on arrival in the recovery room had decreased significantly(p<0.05) to preanesthetic SaO2 2) SaO2 measured in room air had decreased significantly compared with SaO measured in supplemental oxygen. As a result of the study, it is thought to be safe that supplemental oxygen is administered to patient on transfer and in recovery room.
Anesthesia* ; Anesthesia, General ; Anoxia ; Child ; Humans ; Infant ; Masks ; Oxygen* ; Recovery Room ; Respiration

STUDY OBJECTIVES: To identify the value of symptoms in head trauma patients with alert mental status requiring a head CT scan. METHODS: A retrospective study was performed at an emergency center over 8 months period. Patients(age > or = 7) were enrolled if they presented with a Glasgow Coma Scale score of 15 and underwent head CT after head trauma. The symptoms and signs were recorded in the entry forms prior to head CT The symptoms found in the patients with normal CT were compared with those in abnormal CT showing intracranial injuries. RESULTS: Among the 168 patients, traumatic intracranial abnormality was identified in 34(20.2%) by CT. Skull fracture was idendified in 8(4.8%) in normal CT, 17(10.1%) in abnormal CT group by simple X-ray and CT. In the normal CT group without skull fracture, 65(52.0%) had headache, 61(48.8%) had loss of consciousness( LOC), 43(34.4%) had vomiting, 24(19.2%) had nausea, 7(5.6%) had dizziness. In the abnormal CT group without skull fracture, 14(77.8%) had heacache, 9(50.0%) had LOC, 9(50.0%) had vomiting, 2(11.1%) had nausea, 2(11.1%) had dizziness. The patients with headache were significantly more common in the abnormal CT group. CONCLUSION: A head CT is required in alert head trauma patients with any symptoms and signs of intracranial injuries, especially with headache.
Craniocerebral Trauma* ; Dizziness ; Emergencies ; Glasgow Coma Scale ; Head* ; Headache ; Humans ; Nausea ; Retrospective Studies ; Skull Fractures ; Tomography, X-Ray Computed ; Vomiting