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: Recently, increasing attention in the field of dermatological surgery has been paid to treating skin cancers, including both premalignant and malignant lesions. The rhombic flap, one of the transposition flaps, is an outstanding method for reconstructing small- to medium-sized defects after skin surgery. OBJECTIVE: The aim of this study was to evaluate our clinical results with the rhombic flap for reconstruction after Mohs micrographic surgery (MMS), including the cosmetic aspects, complete surgical excision, and recurrence. METHODS: Between June 2010 and September 2013, 37 patients who were diagnosed with premalignant and malignant lesions on the face and extremities were treated with rhombic flaps for the reconstruction of primary cutaneous defects following lesion excisions. We reviewed the medical records and evaluated the clinical aspects and surgical treatment outcomes, and the cosmetic results were scored as excellent, good, fair, or poor. In addition, we assessed the surgical treatment outcomes using the Vancouver Scar Scale (VSS). RESULTS: Thirty-seven patients received 37 rhombic flaps. The cosmetic results of the reconstructions were gratifying, and 28 of 37 patients (75.7%) showed good to excellent results. Specifically, the cosmetic results of the modified rhombic flaps were great, and 27 of 30 patients (90.0%) showed good to excellent results. The cosmetic results on the VSS showed a high mean score (2.9). CONCLUSION: Our study showed that the rhombic flap is a simple reconstruction method and provides aesthetically pleasing results. Therefore, it could be a useful option for reconstructing defects of the face and extremities.
Cicatrix ; Dermatologic Surgical Procedures ; Extremities ; Humans ; Medical Records ; Mohs Surgery* ; Recurrence ; Skin Neoplasms*

BACKGROUND: A collagenoma or connective tissue nevus of the collagen type is a hamartoma consisting predominantly of collagen. Collagenomas are divided into the inherited and acquired types. The acquired forms include eruptive and isolated collagenoma. However, few studies have assessed the characteristics of patients with acquired collagenoma. OBJECTIVE: The aim of this study was to analyze the clinical characteristics of patients with acquired collagenoma. METHODS: We reviewed the medical records and clinical photographs of 23 patients who had been diagnosed histopathologically with acquired collagenoma by biopsy during the last 12 years, from January 2002 to December 2013. In addition, 11 cases of eruptive or isolated collagenomas previously reported in the Korean literature were added. RESULTS: In total, 34 cases of acquired collagenoma were assessed. Of the 34 cases, 53% were men and 47% were women. The mean age at diagnosis was 20.0 years for the patients with eruptive collagenoma and 29.1 years for the patients with isolated collagenoma. In the cases of eruptive collagenoma, multiple, flesh-colored or whitish papules on the trunk were predominant. On the other hand, in the cases of isolated collagenoma, solitary, flesh-colored plaques on the palms or soles were common features. Collagenomas in most cases were asymptomatic (79.4%). However, some patients with collagenomas had other symptoms such as tenderness or pruritus. There was no traumatic history in any case. CONCLUSION: Our study showed that the clinical features of patients with acquired collagenoma share many similarities with those in previously reported studies, with some differences. This study is expected to help us understand and obtain more information on the clinical diagnosis of acquired collagenoma.
Biopsy ; Collagen ; Collagen Type I ; Connective Tissue ; Diagnosis ; Female ; Hamartoma ; Hand ; Humans ; Male ; Medical Records ; Nevus ; Pruritus

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