No abstract available.
Acanthosis Nigricans* ; Growth Hormone*

No abstract available.
De Lange Syndrome*

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

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

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*

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: Herpes zoster (HZ) infection can significantly damage the quality of life of affected individuals and the treatment for HZ is lengthy and expensive. However, there is limited information available on the perception of the disease and the HZ vaccine in Korea. OBJECTIVE: The aim of this study was to determine the awareness of HZ and the HZ vaccine in patients at the dermatology clinic. METHODS: A questionnaire-based survey was conducted with 835 outpatients who visited the dermatologic clinic. RESULTS: The patient responses revealed a high level of awareness of HZ (81.2%), but a lack of detailed knowledge about HZ (56.9%) and the HZ vaccine (39.3%). As a result of this survey, it was confirmed that the level of understanding of the direct cause, pathophysiology, recurrence, and infectiousness of HZ was relatively inadequate, though there was a high level of awareness of the symptoms and clinical features of HZ in the individuals taking the questionnaire. Based on our findings, the knowledge of HZ was higher in females, the elderly, highly educated individuals, and in individuals with multiple chronic diseases (p<0.05). Additionally, the HZ vaccination rate among patients was 3.2%, and the majority of the individuals taking the questionnaire (66.2%) responded that they were willing to get vaccinated based on their physician's recommendation. CONCLUSION: Although the majority of individuals are aware of HZ, they do not appear to have sufficiently detailed knowledge about this condition. Therefore, a nationwide HZ education and vaccination program, in which dermatologists will play a major role, is strongly recommended.
Aged ; Chronic Disease ; Dermatology ; Education ; Female ; Herpes Zoster* ; Humans ; Korea ; Outpatients* ; Quality of Life ; Recurrence ; Vaccination

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: Although several methods have been attempted in the treatment of warts, the traditional destructive method may be painful or ineffective and induce disfiguring scars. Therefore, a more tolerable method with fewer drawbacks is needed. OBJECTIVE: We studied the therapeutic effect of intralesional immunotherapy using the Measles, Mumps, and Rubella (MMR) vaccine and factors affecting it in patients with warts. METHODS: A retrospective study was performed in 207 patients with various types of warts, who did not meet the exclusion criteria for treatment and were followed-up from January 2011 to December 2013. Patients with warts were treated at 2-week intervals, and the therapeutic effect was evaluated 1 month after the final treatment. RESULTS: Overall, 123 (59.5%) patients experienced a greater than half reduction in the size and number of warts. In addition, younger patients (under the age of 20) (p=0.008) and those with common warts (including periungual warts) (p=0.02) showed significantly higher treatment responses. All patients complained of transient mild pain during the intralesional injections, but other side effects were rarely observed. Only 3.2% of patients who experienced a complete response had a recurrence more than 6 months after the final treatment. CONCLUSION: We suggest that intralesional immunotherapy with MMR vaccine can be considered as a primary remedy for patients who are sensitive to pain, especially children with multiple lesions, are concerned about serious side effects, or have common warts, and that treatment response will be improved by increasing the number of applications.
Child ; Cicatrix ; Humans ; Immunotherapy* ; Injections, Intralesional ; Measles* ; Measles-Mumps-Rubella Vaccine ; Mumps* ; Recurrence ; Retrospective Studies ; Rubella ; Rubella Vaccine* ; Warts*