We report a case of Becker's nevus associated with smooth muscle hamartoma, in a 21-year-old male patient, which shows clinically match-head sized, flat topped, round to oval, grouped papules with hairs on the outer surface of the right arm, and microscopically reveals numerous bundles of smooth muscle fiber in the dermis.
Arm ; Dermis ; Hair ; Hamartoma* ; Humans ; Male ; Muscle, Smooth* ; Nevus* ; Young Adult

No abstract available.
B-Lymphocytes ; Lymphoma, B-Cell

No abstract available.
Cavernous Sinus* ; Dura Mater ; Meningitis ; Tuberculosis*

BACKGROUND: Guillain-Barre syndrome (GBS) is subclassified into acute inflammatory demyelinating polyneuropathy, acute motor or motor-sensory axonal neuropathy, and the other variants. While most studies from Western countries report demyelinating type as predominant form of GBS, some reports from Asian countries suggest the opposite result. Because they are not easily discriminated clinically, electrophysiological study is the most practical method for their subclassification. METHODS: Among the patients who had been admitted to our hospital with typical clinical features of GBS over the past 4 years, we analyzed 32 patients, who had undergone at least 2 serial nerve conduction studies (NCS) throughout the course. In each case, conventional NCS in at least 3 limbs were performed and the criteria of Hadden et al., and 2 other criteria were applied. RESULTS: Using the criteria of Hadden et al., 91% of the patients were classified as primary demyelination, and none as primary axonal pathology. Among the cases of primary demyelination, 21% showed electrophysiological features of axonal GBS at least in one occasion during the serial studies. CONCLUSIONS: Our study suggests that the primary demyelinating type is by far more predominant in our population. Secondary changes of electrophysiological findings in some of our cases suggest that primary demyelination could be misinterpreted as primary axonal pathology without timely serial studies. For the epidemiologic study of larger scale in conjunction with clinical features and immunologic investigations, the protocol and criteria of consensus is necessary, with the qualified normal limit values of each laboratory.
Asian Continental Ancestry Group ; Axons ; Classification* ; Consensus ; Demyelinating Diseases ; Epidemiologic Studies ; Extremities ; Guillain-Barre Syndrome* ; Humans ; Neural Conduction ; Pathology

Neurocristopathy is characterized as having a common origin in aberrant neural crest development. Congenital central hypoventilation syndrome (Ondine's curse) is characterized by marked depression of respiratory drive during sleep and normal ventilation while awake because of no response to both hypercapnea and hypoxia. The girl was full-term, weighing 3020 grams. The girl had poor respiratory effort at birth, but improved with oxygen supply and stimulation. abdominal distention and calcification were noted. During laparotomy transitional zone was found at distal jejunum; a jejunostomy was constructed. Numerous attempts at extubation failed because of apnea. The results of an apnea work-up, including brain sonography, echocardiogram, were normal. The girl died of sepsis at 37 days of age. para-aortic ganglioneuroblastoma was found on autopsy. We experienced a newborn with congenital central hypoventilation syndrome, Hirschsprung's disease and congenital ganglioneuroblastoma representative of neurocristopathy.
Anoxia ; Apnea ; Autopsy ; Brain ; Depression ; Female ; Ganglioneuroblastoma* ; Hirschsprung Disease* ; Humans ; Hypoventilation* ; Infant, Newborn* ; Jejunostomy ; Jejunum ; Laparotomy ; Neural Crest ; Oxygen ; Parturition ; Sepsis ; Ventilation

Mitochondrion is an intracellular organelle with its own genome. Its function in cellular metabolism is indispensable that mitochondrial dysfunction gives rise to multisystemic failure. The manifestation is most prominent with tissues of high energy demand such as muscle and nerve. Mitochondrial myopathies occur not only by mutations in mitochondrial genome, but also by defects in nuclear genes or secondarily by toxic insult on mitochondrial replication. Currently curative treatment modality does not exist and symptomatic treatment remains mainstay. Administration of L-arginine holds great promise according to the recent reports. Advances in mitochondrial RNA import might enable a new therapeutic strategy.
Arginine ; Genome ; Genome, Mitochondrial ; MELAS Syndrome ; MERRF Syndrome ; Mitochondria ; Mitochondrial Myopathies ; Muscles ; Ophthalmoplegia, Chronic Progressive External ; Organelles ; RNA

No abstract available.
Child* ; Humans ; Hydronephrosis*

Metastases to the skin from bladder carcinoma are very the rare and rate is 1 to 2% of the cutaneous metastases. A 61-year-old man presented with 4 months history of well demarcated erythematous eroded nodules on the both inguinal areas which developed 3 years later after the diagnosis of carcinoma of the urinary bladder had been made. Histological examination of the skin lesion revealed atypical cells having hyperchromatic and pleomorphic nuclei which were similar to those of the urinary bladder. We report herein a case of cutaneous metastasis from carcinoma of the urinary bladder in a 61-year-old man.
Cytochrome P-450 CYP1A1 ; Diagnosis ; Humans ; Middle Aged ; Neoplasm Metastasis* ; Skin ; Urinary Bladder*

BACKGROUND: Food allergies have been demonstrated to play an important role in the pathogenesis of atopic dermatitis(AD), affecting around 10 to 40% of patients with AD. While immediate-type clinical reactions to food can easily be identified, the evaluation of a food allergy in the absence of immediate clinical reactions still presents diagnostic difficulties. OBJECTIVE: The purpose of this study was to evaluate the diagnostic value of the atopy patch test with regard to late-phase reactions and to evaluate the relationship between patch test and specific serum IgE level. METHODS: We performed the patch test and chemiluminescent assay(CLA) for food antigens(cow's milk, soybean milk and hen's egg) in 45 patients with AD who were suspicious of food-related symptoms and 15 normal controls. RESULTS: 1. Positive reaction of CLA in 45 patients with AD were 7/18(38.9%) for the cow's milk, 5/14(35.7%) for the soybean milk and 4/13(30.7%) for the hen's egg. 2. Positive reaction of the patch test in patients with AD were 5/18(27.8%) for the cow's milk, 6/14(42.9%) for soybean milk and 4/13(30.7%) for hen's egg, but only one(6.7%) for cow's milk in 15 normal controls. 3. A highly significant correlation to food antigens existed between patch test and CLA, but one patient showed a positive reaction in the patch test for soybean milk despite the negative reaction in CLA. 4. There was no significant statistical correlation between the severity of AD and the positive reaction rate of the patch test. 5. There were no significant statistical differences between the age of AD patients and the positive reaction rate of the patch test. CONCLUSION: The atopy patch test seems to be a valuable tool in the diagnostic work-up of food allergies in patients with AD-especially with regard to late-phase clinical reactions.
Dermatitis, Atopic* ; Food Hypersensitivity ; Humans ; Immunoglobulin E* ; Luminescent Measurements ; Milk ; Ovum ; Patch Tests* ; Soybeans

Sebaceous carcinoma is a malignant tumor derived from the adnexal epithelium of sebaceous glands and is usually located on the meibomian glands of the eyelid, but may occur on the extraocular sites. We present two cases of sebaceous carcinoma developed on the unusual site. The first case was 66-year old woman, who had 2.2X3.0cm sized, yellowish, verrucous surfaced plaque on the abdomen for 20 years. The second case of 79-year old man had annular shaped erythematous greasy patch with peripherally located flat plaque on the left scapular area for 1 month. His past medical history included subtotal gastrectomy for early gastric cancer(type IIa) and could not find any recurrence or metastasis for 4 years duration. The histopathologic examination revealed irregular, variable sized tumor lobules. Each lobule was composed of sebaceous and undifferentiated cells showing considerable variation in the shape and size of their nuclei and prominent nucleoli. On the basis of clinicopathologic examination we diagnosed first case as sebaceous carcinoma and second case as Muir-Torre syndrome.
Abdomen ; Aged ; Epithelium ; Eyelids ; Female ; Gastrectomy ; Humans ; Meibomian Glands ; Muir-Torre Syndrome* ; Neoplasm Metastasis ; Recurrence ; Sebaceous Glands