BACKGROUND: The common histopathology of Behget's disease is vasculitis associated with activation of neutrophils. The level of plasma elastase a 1 proteinase inhibitor (E a 1 PI), which represents the activation of neutrophils, may be a marker of Behcet's disease. OBJECTIVE: We examined the level of plasma elastase al proteinase inhibitor to evaluate the degree of neutrophil activation in Behcet's disease. METHOD: We measured plasma elastase a 1 proteinase inhibitor in 34 cases of untreated Behcet's disease patients and 30 cases of normal individuals by an enzyme immunoassay. We also studied the differences between the levels in two clinical types of Behcet's disease, the complete and incomplete type. RESULTS: The plasma level of elastase a 1 proteinase inhibitor was significantly higher in untreated Behcet's disease patients than in healthy controls. However, there was no significant difference between the levels in the two clinical types. CONCLUSION: These data suggest that the elevated level of plasma elastase a 1 proteinase inhibitor may reflect a state of chronic activation of neutrophils in Behcet's disease immunologically and further studies will be needed to evaluate the clinical status of Behcet's disease patients by measuring levels of plasma elastase a 1 proteinase inhibitor.
Humans ; Immunoenzyme Techniques ; Methods ; Neutrophil Activation ; Neutrophils ; Pancreatic Elastase ; Plasma* ; Vasculitis

Atypical absence is less understood than typical absence. Several conditions that produce atypical absence are known including Lennox-Gastaut syndrome, myoclonic astatic epilpsy and epileptic encephalopathy with continuous spike and waves in slow wave sleep. A 17-year-old girl with mental retardation had developed frequent loss of consciousness and occasional falling attack with traumatic facial injury for 2 years. The interictal EEG showed 2 Hz slow spike-and-wave complex with maximum over right frontotemporal area and the brain MRI was normal. Carbamazepin was prescribed initially but the drug seemed to worsen the seizures. Long term video-EEG monitoring showed very frequent atypical absence seizures consisting of sudden hypotonia of head and oral automatism with or without secondary generalization. Generalized 2 to 2.5 Hz slow spike-and-wave complexes with duration of 10 to 40 seconds were seen during ictal period. About 10% to 20% of the non REM sleep was occupied with generalized slow spike-and-wave complex and/or polyspikes or polyspikes-and-wave complex with duration of within 1 second. Valprorate monotherapy had failed, then lamotrigin was added. In spite of polytherapy, the seizure was intractable. We think this intractable atypical absence might be associated with juvenile onset Lennox-Gastaut syndrome.
Adolescent ; Automatism ; Brain ; Electroencephalography ; Epilepsy, Absence ; Facial Injuries ; Female ; Generalization (Psychology) ; Head ; Humans ; Intellectual Disability ; Magnetic Resonance Imaging ; Muscle Hypotonia ; Seizures ; Sleep, REM ; Unconsciousness

No abstract available.
Gastrointestinal Tract* ; Leiomyosarcoma*

No abstract available.
Colorectal Neoplasms* ; Fluorouracil* ; Leucovorin*

As a rule, eccrine spiradenoma occurs as a solitary intradermal nodule measuring 1 to 2 cm in diameter. Occasionally, there are several nodules, and rarely, there are numerous small nodules in a zosteriform pattern or large nodules, up to 5 cm, in a linear arrangement. We report herein an interesting case of eccrine spiradenoma in which multiple large nodules in a linear arrangement were observed. A 41-year-old female presented with a 17-year history of multiple pea to large bean-sized painful and tender subcutaneous nodules which show the linear distribution along her right arm. The diagnosis of eccrine spiradenoma was made by clinical and histopathological findings and all her subcutaneous nodules were removed by surgical method.
Adult ; Arm ; Diagnosis ; Female ; Humans ; Peas

BACKGROUND: Graying of hair is usually a manifestation of the aging process and is due to a progressive reduction of the activity of melanocytes in the hair bulb. OBJECTIVE: We performed this study to investigate the clinical features of the graying process in Korea.ns. METHOD: The study population consisted of 1,058, comprising 589 men and 469 women between the fourth and seventh decade. They were studied statistically by using questionaires about graying and 12 persons with gray hair were observed clinically on the grouping pattern of scalp hairs by the dermatologist. RESULT: 1. More than 50 percent of the population over 40 years old had gray hair on the scalp. 2. The age of onset of graying was variable but generally between 35 and 44 years. 3. The temporal area of the scalp was the most common site of initial graying and the most abundant site of gray hair. 4. There were no relationship between gray hair and male pattern baldness. 5. Generally each one of the hairs in the grouped hair became gray independently, not in union. 6. The proportion of graying in the grouped hair was higher than that of the single hair. CONCLUSION: According to the results of the study, more than 50 percent of the population over 40 years of age have gray hair and the temporal area usually becomes gray first and is the most abundant site of gray hair. Further studies will be needed in regard to the graying in the grouped hair, though we think that each one of the hairs in the grouped hair may become gray independently and the graying process of the grouped hair may occur earlier than that of the single hair.
Adult ; Age of Onset ; Aging ; Alopecia ; Female ; Hair* ; Humans ; Male ; Melanocytes ; Scalp

The Dowling-Meara type of epidermolysis bullosa simplex is a rare genodermatosis transmitted by autosomal dominant pattern. It starts during the first 3 months of life and is characterized by generalized bullae in a herpetiform pattern. We report two cases of Dowling-Meara type of epidermolysis bullosa simplex. The first case is an 18-year-old male patient who showed bullae in a herpetiform arrangement on the dorsa of both hands, forearms, and trunk, hyperkeratosis on the palms and soles, postinflammatory hypo- and hyperpigmentations, and dystrophy of finger and toe nails. The second case is a 1-month-old boy who showed multiple bullae in a herpetiform and confluent arrangement scattered on the whole body, particularly on the dorsa of both feet and hands. Microscopically and electro-microscopically, two cases all showed intraepidermal blisters formed within the basal keratinocyte in association with clumping of the tonofilaments and cytolysis of the basal cells.
Adolescent ; Blister ; Epidermolysis Bullosa Simplex* ; Epidermolysis Bullosa* ; Fingers ; Foot ; Forearm ; Hand ; Humans ; Infant, Newborn ; Intermediate Filaments ; Keratinocytes ; Male ; Toes

BACKGROUND: Strategic infarct dementia (SID) is characterized by focal ischemic lesions involving specific sites that are critical for higher cortical functions. However, the mechanisms of SID are not well understood. We evaluated lesion sites, neuropsychiatric symptoms, neuroimaging and neuropsychological findings in patients with SID and have come up with suggestions of the mechanism behind SID. METHODS: Eleven patients with SID according to the NINDSAIREN criteria for vascular dementia were included. All patients were given a neurologic examination, brain MRI with MRA and brain perfusion SPECT using Tc-99m HMPAO. We evaluated neuropsychiatric symptoms and neuropsychological status using a Korean-version of the Neuropsychiatric Inventory(K-NPI) and Seoul Neuropsychological Screening Battery. RESULTS: Various sites were responsible for SID; the thalamus (n=5), genu of internal capsule (n=2), temporooccipital lobe (n=2), medial temporal lobe (n=1), medial frontal lobe (n=1). The most common neuropsychiatric symptom was apathy according to the K-NPI. Brain perfusion SPECT revealed ipsilateral cortical hypoperfusion, mainly in the frontal and temporal areas. In several cases, there were some degrees of cortical hypoperfusion in the contralateral areas of the lesion. On neuropsychological assessment, cognitive deficits on attention and frontal executive function were prominent. CONCLUSIONS: The thalamus, genu of internal capsule, and temporooccipital area were the most common sites responsible for SID. Based on the results that there were cortical hypoperfusion ipsilateral to subcortical strategic infarct and prominent cognitive deficits on attention and frontal executive function, it is suggested that disruption of the frontal-subcortical circuit may play an important role in patients with SID.
Apathy ; Brain ; Cerebral Infarction ; Dementia* ; Dementia, Vascular ; Executive Function ; Frontal Lobe ; Humans ; Internal Capsule ; Magnetic Resonance Imaging ; Mass Screening ; Neuroimaging* ; Neurologic Examination ; Neuropsychological Tests ; Perfusion ; Seoul ; Sudden Infant Death ; Technetium Tc 99m Exametazime ; Temporal Lobe ; Thalamus ; Tomography, Emission-Computed, Single-Photon

Carcinoid tumors are derived from enterochromaffin cells that are capable of producing a wide range of neuroendocrine mediators including serotonin or 5-hydroxytryptamine. Carcinoid syndrome occurs when mediators produced by the tumor and normally metabolized by the liver escape into the systemic circulation. The syndrome classically involves the gastrointestinal tract, respiratory system, cardiovascular system and the skin. Flushing is almost universal in the syndrome. A 23-year-old woman came to our hospital presenting with flushing on face and trunk. The patient had experienced flushing for 2 years and it was aggravated by emotional change, stress, exercise, and eating spicy food. The patient also had abdominal pain, diarrhea, weight loss and hepatomegaly. Urinary 5-hydroxyindoleacetic acid was elevated (81.5 mg/day). Abdominal computed tomography scans showed multiple hepatic masses diagnosed as a carcinoid tumor by computed tomography-guided needle biopsy. Physicians should consider carcinoid syndrome when patients present with flushing and systemic symptoms.
Abdominal Pain ; Biopsy, Needle ; Carcinoid Tumor ; Cardiovascular System ; Collodion ; Diarrhea ; Eating ; Enterochromaffin Cells ; Female ; Flushing ; Gastrointestinal Tract ; Hepatomegaly ; Humans ; Liver ; Porphyrins ; Respiratory System ; Serotonin ; Skin ; Skin Manifestations ; United Nations ; Weight Loss ; Young Adult

BACKGROUND: Vitiligo, a disease presenting as white patches on the skin, is caused by selective destruction of melanocytes, which are completely absent in established lesions. There are many theories about the etiology of vitiligo including the self-destruct, biochemical, neural, autoimmune and genetic hypotheses. Oxidative stress has been implicated in vitiligo as a causative factor. OBJECTIVE: To examine the susceptibility of vitiligo keratinocytes to external oxidative stress, H2O2, we compared survival rates in cultured keratinocytes according to the different concentration and exposure time of H2O2. METHODS: Keratinocytes were prepared from normal control volunteer, and the center of lesion and non-lesional skin of vitiligo patients. It was evaluated in cultures at the second and third passages. And, keratinocytes were exposed to various concentrations of hydrogen peroxide (H2O2) for 10, 20 and 40 minutes. Finally, the 96 wells plate containing cells were performed MTT assay. RESULTS: Compared to normal keratinocytes, vitiligo keratinocytes, both non-lesional and lesional, showed markedly lower survival rate. At the concentration of 0.01, 0.1 and 1 mM H2O2, survival rates did not significantly affect viability of keratinocytes after neither exposure times. On the contrary, vitiligo keratinocytes, both non-lesional and lesional, were more susceptible to the toxic effect of H2O2 after the period of exposure at concentration of 10mM H2O2 compared with normal keratinocytes. The survival rate of at concentration of 10mM H2O2 inversely correlated with the exposure period in all experiments. CONCLUSIONS: Our results demonstrate the presence of an imbalance in the anti-oxidant system in vitiligo keratinocytes and provide further support for oxidative damage as a pathogenic event in vitiligo.
Humans ; Hydrogen Peroxide ; Keratinocytes* ; Melanocytes ; Oxidative Stress* ; Skin ; Survival Rate ; Vitiligo* ; Volunteers