No abstract available.
Acanthosis Nigricans* ; Growth Hormone*

A 3-year-old-male had the appearance of red urine at birth and developed recurrent bullae in sun-exposed area of the skin, erythrodontia, alopecia, splenomegaly and hemolytic anemia, We observed coral red fluorescence of the teeth and urine under Wood's light and detected excessive excretion of the uroporphyrin in the urine and coproporphyrin in the stool wlth inreased porphyrin in the blood. Fluorescence of erythrocyte was demonstrated by:fluoreacence microscopy. Histologic findings showed subepidermal bulla with PAS-positive hyaline deposits around the blood vessels and revealed IgG deposits in the wall of blood vessels and dermo-epidermal junction by direct immunofluorescence.
Alopecia ; Anemia, Hemolytic ; Anthozoa ; Blood Vessels ; Erythrocytes ; Fluorescence ; Fluorescent Antibody Technique, Direct ; Hyalin ; Immunoglobulin G ; Microscopy ; Parturition ; Porphyria, Erythropoietic* ; Porphyrias ; Skin ; Splenomegaly ; Tooth

No abstract available.
De Lange Syndrome*

Drug-induced toxic epidermal necrolysis (TEN) is a bullous erythematous disease that is characterized by the appearance of scaled lesions and large sheets of pilling on the skin. A caseof TEN occured in a 10 month old female patient. The characteristic skin lesions of TEN developed after oral administration of carbamazepine for a partial seizure. Thig case consists of prodrome of malaise, fever, anorexia, and conjunctivity followed by erythema & flaccid bullae formation. Diagnosis was confirmed by histologic findings. This patient was treated with a massive systemic corticosteroid, antibiotics, fluid and electrolytes, and topical measures. Authors experienced an extremely rare case of TEN dur to carbamazepine, So report it with a brief review of literature.
Administration, Oral ; Anorexia ; Anti-Bacterial Agents ; Carbamazepine ; Diagnosis ; Electrolytes ; Erythema ; Female ; Fever ; Humans ; Infant ; Seizures ; Skin ; Stevens-Johnson Syndrome*

The Kniest syndrome is characterized by disproportionate dwarfism and Kyphoscoliosis which may be associated with flat facies with prominent eyes, cleft palate, hearing loss, myopia and limited joint motion. The skeletal abnormalities are recognizable at birth with shortening and deformity of the extremities and stiff joints. Marked lumbar lordosis and kyphoscoliosis develop in childhood, resulting in disproportionate shortening of the trunk. We experienced a case of kniest syndrome, confirmed by clinical features, radiological features, and histological examination of cartilage. A brief review of the related literature is presented.
Animals ; Cartilage ; Cleft Palate ; Congenital Abnormalities ; Dwarfism ; Extremities ; Facies ; Hearing Loss ; Joints ; Lordosis ; Myopia ; Parturition

BACKGROUND: Initial symptoms for esophageal perforation have not been clarified, but when there is no early diagnosis and proper treatment to follow immediately after the diagnosis, it is fatal for the patients. Therefore, this study attempted to discover the factors that influence the prognosis of esophageal perforation to contribute to the improvement of the treatment result. MATERIAL AND METHOD: The subjects of this study are 32 patients who came to the hospital with esophageal perforation from October, 1984 to June, 2000. This study examined the items for clinical observation such as patients' sex, age, cause of the perforation, perforation site, the time spent until the beginning of the treatment, symptoms caused by the perforation and its complication, and treatment methods. This study tried to find out the relationship between the survival of patients and each item. RESULT: There were 24 male and 8 female patients and their mean age was 49.7 +/- 16.4. For the causes of perforation, there were 14 cases(43%) of iatrogenic perforation, which ranked first, caused by the medical instrument operation and surgical damage. As for the perforation sites, thoracic esophagus was the most common site(26 cases of 81.2%) and chest pain was the most frequent symptom. The complication caused by esophageal perforation showed the highest cases in the order of mediastinitis, empyema, sepsis and peritonitis. After the treatment, there were 23 cases of survival and 9 cases of mortality. The total mortality rate was 28.1% and the main causes of mortality were sepsis and acute respiratory distress syndrome(ARDS). As for the treatment, 8 cases(25.0%) treated the perforation successfully using conservative treatment only. As for the surgical treatment, there were 5 cases(15.6%) of cervical drainage, 7 cases (21.8%) of primary repair and 12 cases(37.5%) of esophageal reconstruction after performing an exclusion-diversion. There were 18 cases(56.2%) of complete treatment of esophageal perforation at its initial treatment and in 14 cases(43.8%) of treatment failure at its initial treatment, patients were completely cured in the next treatment stage or died during the treatment. The cases of perforation in thoracic esophagus, complication into severe mediastinitis or sepsis and the cases of failure at initial treatment showed a statistically significant mortality rate (p<0.05). CONCLUSION: Based on the above results, it is thought that a proper choice for initial treatment choice depending on the perforation site and the prevention of serious complication such as mediastinitis or sepsis can shorten the treatment period for the patients with esophageal perforation and improve the convalescence.
Chest Pain ; Convalescence ; Diagnosis ; Drainage ; Early Diagnosis ; Empyema ; Esophageal Perforation* ; Esophagus ; Female ; Humans ; Male ; Mediastinitis ; Mortality ; Peritonitis ; Prognosis ; Sepsis ; Treatment Failure

BACKGROUND: Epidermal cyst is a common acquired skin cyst. When such cysts may be inflamed, they are often referred to as being infected. To clarify the etiology of inflamed epidermal cysts, several studies have carried bacteriology of inflamed and uninflamed epidermal cyst and sought to identify the role of micro-organisms. OBJECTIVE: The aim of this study is to evaluate the bacterial influences on inflammatory process of epidermal cysts and the antibiotic sensitivity of cultured aerobic bacteria in epidermal cysts. METHODS: We carried out the bacterial cultures using sterile swabbing internal materials in each cyst which has been incised after surgical removals of epidermal cyst. An inflamed epidermal cyst was defined as a known cyst that developed a fluctuant soft-tissue swelling surrounded by the erythema and contained a localized collection of purulent material. For the aerobic culture, samples were cultured on blood agar plates, McConkey agar plate and chocolate agar plates in 5% CO2 at 35degrees C for 5 days. Colonies formed were identified based on VITEK2 system. Then antimicrobial susceptibility test were also done on VITEK2 system. RESULTS: Total of 100 epidermal cyst specimens from 96 patients (67 men and 29 women) were involved had confirmed histopathological findings by dermatologists. Seventy were from face and neck, 24 from trunk, 4 from the extremities, 2 from buttock. Of the 53 inflamed cysts, 30 (56.6%) yielded bacterial growth. On the other hand, from the 47 uninflamed cysts, 15 (31.9%) cyst resulted in bacterial growth (p=0.013). The predominant bacteria from inflamed and uninflamed cyst were coagulase-negative Staphylococci (32 isolate of 45 specimens, 71.1%). All cultured bacteria were susceptible to almost all of antibiotics except amoxicillin/clavulanic acid, ampicillin, benzylpenicillin, cefoxitin, cephalothin, fucidic acid, piperacillin, piperacillin/Tazobactam. CONCLUSION: Our study showed that CoNS was predominant in inflamed cysts, which strongly suggests that aerobic bacteria play a role in the inflammatory process and treatment with antibiotics is necessary for epidermal cyst.
Agar ; Ampicillin ; Anti-Bacterial Agents ; Bacteria ; Bacteria, Aerobic ; Bacteriology ; Buttocks ; Cacao ; Cefoxitin ; Cephalothin ; Epidermal Cyst ; Erythema ; Extremities ; Hand ; Humans ; Male ; Neck ; Penicillin G ; Piperacillin ; Skin

Hand-foot-mouth disease (HFMD) is an infectious viral disease that is common among children. It is clinically characterized by vesicular eruptions on the palms and soles and a maculopapular rash. Onychomadesis is a periodic idiopathic shedding of the nails at their proximal ends and results from arrest of the proliferative function of the nail matrix. Recently, a few reports described onychomadesis following HFMD, although the mechanism remains unclear. To our knowledge, this association has not been reported in Korea. Herein, we report two cases of onychomadesis following HFMD and review the published data.
Child ; Exanthema ; Humans ; Korea ; Virus Diseases

Dermoscopy is a noninvasive technique that is known to increase the accuracy of diagnosing benign versus malignant skin lesions. Milia en plaque is a rare benign epidermal tumor characterized by multiple milia-like lesions within the erythematous edematous base. It arises spontaneously on healthy skin in predisposed individuals without any apparent cause. An 80-year-old woman presented with a solitary coin-sized yellowish to brown plaque on the right cheek. The lesion has been enlarging for several years. Dermoscopic examination showed arborizing telangiectasia, and we suspected it to be basal cell carcinoma. Two biopsies were taken from the lesion, which revealed a keratin-filled cyst lined by a stratified epithelium of a few cell layers and sparse lymphocytic infiltration. Therefore, we diagnosed the lesion as milia en plaque, histopathologically. On the basis of our experience with a case of milia en plaque diagnosed as a basal cell carcinoma with dermoscopic findings, we believe that benign lesions should be considered even in the presence of malignant findings on dermoscopy.
Aged, 80 and over ; Biopsy ; Carcinoma, Basal Cell* ; Cheek ; Dermoscopy ; Epithelium ; Female ; Humans ; Skin ; Telangiectasis

BACKGROUND AND OBJECTIVES: The aims of this study were to evaluate the anatomical features of patients with obstructive sleep apnea syndrome (OSAS) using upper airway computed tomography (CT) in the awake state, and to analyze the correlation between anatomical features and severity of polysomnography (PSG) findings. MATERIALS AND METHODS: Sixty-eight patients presenting with snoring and sleep apnea were included, and upper airway CT in the awake state and PSG were performed in all patients. The average apnea-hypopnea index (AHI) and minimal arterial oxygen saturation (minSaO2) values were calculated. The axial and sagittal images from the level of the nasopharynx to that of the hypopharynx were obtained and measured. The correlations of the anatomical parameters with AHI and minSaO2 were statistically analyzed. RESULTS: The significant parameters correlated with AHI were nasopharynx anterior-posterior (A-P) diameter and transverse diameter, soft palate transverse diameter, uvula length, tongue length, lingual transverse diameter, and hypopharynx A-P diameter & transverse di-ameter. The parameters correlated with minSaO2 were nasopharynx transverse diameter and lingual transverse diameter. Conclusions: Upper airway analysis using CT in awake OSAS patients provides anatomical parameters which correlate with the severity of OSAS. Therefore, upper airway CT in the awake state may be helpful in diagnosing OSAS.
Airway Obstruction ; Humans ; Hypopharynx ; Nasopharynx ; Oxygen ; Palate, Soft ; Polysomnography ; Sleep Apnea Syndromes ; Sleep Apnea, Obstructive ; Snoring ; Tongue ; Uvula ; Wakefulness