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: 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

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: The anatomical relation between a malignant tumor and its vascular and lymphatic bed is an important influencing metastasis. Hox D3 is required for these expressions of integrin alpha v beta3 and urokinase plasminogen activator (uPA), which contribute to endothelial cell adhesion, invasion, and migration during angiogenesis. Recent studies in different tumor types have shown that vascular endothelial growth factor-C (VEGF-C), which displays a high specificity for lymphatic endothelium, is involved in tumor-induced lymphagiogenesis and lymphatic metastatic spread. OBJECTIVE: This study was designed to measure the expression of HOX D3 and VEGF-C in different skin cancers. METHODS: The expression of HOX D3 and VEGF-C was examined by immunohistochemical staining of 40 skin cancer tissue samples, including 8 keratoacanthomas, 8 extramammary paget's disease, 8 basal cell carcinomas, 8 squamous cell carcinomas and 8 malignant melanomas. RESULTS: Immunohistochemical analysis of 40 skin cancer tissue samples revealed a high expression of HOX D3 and VEGF-C in the more aggressive and invasive skin tumors, including squamous cell carcinomas and malignant melanomas. On the other hand, low expression was seen in the less-invasive skin tumors, including keratoacanthomas, extramammary paget's disease and basal cell carcinomas. Also the degree of expression of HOX D3 and VEGF-C showed a statistically-significant correlation with each skin tumor (p<0.05). CONCLUSION: These findings provide evidence that the upregulation of HOX D3 and VEGF-C might be involved in the promotion of angiogenesis and lymphagiogenesis in skin tumors and play an important role in metastasis.
Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Endothelial Cells ; Endothelium, Lymphatic ; Hand ; Integrin alphaV ; Keratoacanthoma ; Lymphangiogenesis ; Melanoma ; Neoplasm Metastasis ; Paget Disease, Extramammary ; Plasminogen Activators ; Sensitivity and Specificity ; Skin Neoplasms ; Skin* ; Up-Regulation ; Urokinase-Type Plasminogen Activator ; Vascular Endothelial Growth Factor C*

Malignant Peripheral Nerve Sheath Tumor is a neoplasm that most often arises in anatomically discernible peripheral nerves or neurofibroma. This rare tumor usually has a strong association with neurofibromatosis type 1. Nearly half of these malignant tumors arise in individuals affected with neurofibromatosis type 1. Neurofibromas often first appear around the time of puberty, increase in size and number during pregnancy, and shrink after giving birth. Because growth hormone (GH) concentration increases during puberty and pregnancy, it is possible that GH influences the growth of these tumors. A 33-year-old pregnant woman presented with a 4-month history of a painful, huge mass growing on the right breast. She suffered from neurofibromatosis type 1, with multiple cafe au lait spots and neurofibromas. The clinical and histopathological findings showed a malignant peripheral nerve sheath tumor. We report a case of malignant peripheral nerve sheath tumor on the right breast in a pregnant woman with neurofibromatosis type 1.
Adolescent ; Adult ; Breast ; Cafe-au-Lait Spots ; Female ; Growth Hormone ; Humans ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1 ; Parturition ; Peripheral Nerves* ; Pregnancy* ; Pregnant Women ; Puberty