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

Lithium salts are being used increasingly to treat patient with affective disorders, especially acute mania, or bipolar manic-depressive illness. For therapeutic effect the lithium content must be maintained at or above a particular level. Lithium poisoning due to overdosage may be seen occasionally, and its course is determined primarily by the rate of renal lithium elimination. A search is therefore indicated for procedures that could raise the lithium clearance. In a number of reports renal lithium excretion has been studied in relation to the excretion of water, sodium, potassium and hydrogen, but effects of sodium or water on the lithium excretion has not yet been clarified. Hence the present study was undertaken to investigate the effects of corticosteroid on the excretion of lithium ion. The female rat (Sprague-Dowley), weighing from 200 to 300g, was injected with 50mg/kg of lithium chloride intraperitoneally, and then injected with graded dosage of fludrocortisones and dexamethasone in each group. During the injected rats were incubated in metabolic cage, 24 hour urine of rats were collected. At 24 hours after injection, the rats were sacrificed with guillotine, the blood were collected. And then the concentrations of Na⁺, K⁺, Li⁺ of collected urine and serum were checked by Flame photometer. The results are summarized as follows 1. Fludrocortisone decreased the serum concentration of lithium and increased the urinary excretion of lithium. 2. In the group treated with low dose of dexamethasone (0.1 mg/kg), the serum concentration of lithium was decreased and high dose of dexamethasone (1 mg/kg) increased the urinary excretion of lithium. 3. Fludrocortisone increased the urinary [Na⁺]/[K⁺] in serum and decreased [Na⁺]/[K⁺] inurine, but opposite effects were occurred in dexamethasone. By above results, it may be concluded that corticosteroid increased the urinary excretion of lithium and decreased the serum concentration of lithium, but it seems to be there in no relationship between these effects of corticosteroid and of the renal Na⁺ or K⁺ transport.
Adrenal Cortex Hormones* ; Animals ; Bipolar Disorder ; Dexamethasone ; Female ; Fludrocortisone ; Humans ; Hydrogen ; Lithium Chloride ; Lithium* ; Mood Disorders ; Poisoning ; Potassium ; Rats ; Renal Elimination* ; Salts ; Sodium ; Water

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

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*

Localized pulmonary amyloidosis is a rare disease characterized by a deposition of amyloid limited to the lungs. We report a case of primary tracheobronchial amyloidosis which resulted in collapse of left lung and was confirmed by the Congo-red staining.
Amyloid ; Amyloidosis* ; Lung ; Rare Diseases