Neurofibromatosis is a systemic hereditary disorder with varied manifestations in bone, soft tissue, the nervous system, and skin. Neurofibromatosis is characterized by cafe au lait macules, neurofi- bromas, Lisch nodules, optic gliomas, bony dysplasia, intertriginous freckling, and autosomal inheritance. Juvenile xanthogranuloma(JXG) is a benign, self-healing disorder of infants, children, and occa- sionally adults, characterized by yellowish papulonodular lesions located in the skin and other organs and consisting of an infiltrate of histiocytes with a progressively greater degree of lipidation in the absence of metabolic disorders. In 1954 Normland reported the first case of a JXG in a child with numerous cafe au lait macules. Royer, in 1958, reported the triple association of JXG, neurofibromatosis, and leukemia. It is estimated that children with neurofibromatosis and JXG have a higher risk for leukemia than do patients with neurofibromatosis who do not have JXG. In patients with both JXG and neurofibromatosis, long-term follow-up is required because of the additional association with leukemia. We report a case of JXG associated with neurofibromatosis.
Adult ; Child ; Follow-Up Studies ; Histiocytes ; Humans ; Infant ; Leukemia ; Nervous System ; Neurofibromatoses* ; Neurofibromatosis 1 ; Optic Nerve Glioma ; Skin ; Wills ; Xanthogranuloma, Juvenile*

In clinical setting, treatment-refractoriness, medication induced tardive dyskinesia and amenorrhea in chronic schizophrenia are frequently problematic. However, there are few guideline solving these problem available to clinicians. The goal of this study was collecting clinical data on clinical effeciveness and predictors of response of switching to olanzapine. We attempted to switch to olanzapine from risperidone and clozapine in chronic 31(risperidone 17, clozapine 14) schizophrenia and schizoaffective disorder patients suffering from sustained symptoms, weekly blood monitoring, medication induced tardive dyskinesia and amenorrhea. Previous antipsychotics dosage was gradually decreased for 2 or 3 weeks, at the same time olanzapine dosage was gradually increased. At baseline, after 1 week, after 2 weeks and after 4 weeks we checked Brief Psychiatric Rating Scale, Clinical Global Impression Scale, Sympson-Angus Rating Scale, Barnes Akathisia Rating Scale and Followed up after 12 months. Successful switch after 4 weeks was achieved in 25 patients(clozapine 9(64.2%), risperidone 16(94.1%). Overall, mean BPRS and CGI scores increased significantly. Successful maintenance after 12 months was achieved in 17 patients(clozapine 5(35.7%), risperidone 2(70.5%). Overall, mean BPRS and CGI scores increased significantly too. Switching to olanzapine from other atypical antipsychotics is recommendable in chronic schizophrenia with treatment refreactoriness and drug induced side effect.
Amenorrhea ; Antipsychotic Agents ; Brief Psychiatric Rating Scale ; Clozapine* ; Female ; Follow-Up Studies* ; Humans ; Movement Disorders ; Psychomotor Agitation ; Psychotic Disorders ; Risperidone* ; Schizophrenia

Short nose is characterized by decreased distance from the nasion to tip defining point and increased nasolabial angle with increased nostril show. Lengthening the short nose is arguably the most difficult operation in aesthetic rhinoplasty. We have described here a simple and easy technique that lengthen the short nose by using silicone implant has been popular among Asian for augmentation rhinoplasty. Through open rhinoplastic approach, wide undermining of the nasal skin is performed to allow redraping of the skin over the lengthened skeletal framework without excessive tension. Alar cartilage is released from upper lateral cartilage and relocated caudally. After relocation of alar cartilage, silicone implant is inserted in subperiosteal pocket and immobilized by nonabsorbable suture material at dorsal aspect of septal cartilage. Narrow caudal end of implant is sutured between medial crura of alar cartilage which were caudally rotated. This implant not only raise the nasion but can move the tip-defining point caudally by keeping the alar cartilage in new position. Tip graft is done with conchal cartilage. We have got the satisfactory result in cosmetic aspect without any complications.
Asian Continental Ancestry Group ; Cartilage ; Humans ; Nose* ; Rhinoplasty ; Silicones* ; Skin ; Sutures ; Transplants

Dermal melanocytosis is characterized by the presence of ectopic melanocytes in the dermis and shows several morphological forms, including the mongolian spot, the blue nevus, the nevus of Ota, the nevus of Ito, and dermal melanocyte hamartoma. A 30-month-old boy had a gray blue patch on the left deltoid region, upper back, sacral area, the entire length of the right arm, and right hand. There was a bluish speckled patch on the right palm. The lesions were present at birth, and no appreciable change in appearance had occurred. He also had blue to brownish pigmentation on the right side of his face with dark bluish pigmentation of the right sclera. His Mental and physical development had been normal. The histopathological examination revealed dendritic dermal melanocytes, mainly in the upper and mid dermis. Clinically and pathologically, the patient was diagnosed as having Ota nevus, Ito nevus, mongolian spot, and dermal melanocyte hamartoma. To our knowledge, dermal melanocytosis with 4 different morphological forms in a patient has not previously been reported. We report this unique case of dermal melanocytosis with various morphological forms.
Arm ; Child, Preschool ; Dermis ; Hamartoma ; Hand ; Humans ; Male ; Melanocytes ; Mongolian Spot ; Nevus ; Nevus of Ota ; Nevus, Blue ; Parturition ; Pigmentation ; Sclera

Eosinophilic gastroenteritis is characterized by peripheral eosinophilia, eosinophilic infiltration of the bowel and gastrointestinal symptoms. The disease may affect any area of the gastrointestinal tract. Various manifestations are present, corresponding to the predominant layer of the eosinophilic infiltration. We experienced a case of eosinophilic gastroenteritis presenting as protein-losing enteropathy in a 36-year old male patient with acute upper abdominal pain and watery diarrhea. He showed peripheral eosinophilia, hypoalbuminemia, penile., thigh and ankle edema. The patient was treated with oral corticosteroid, and supportive care such as parenteral albumin, fluid and electrolytes. Almost all symptoms resloved within 1 week. The present report concerns a case of eosinophilic gastroenteritis associated with protein loss. Cases, of eosinophilic gastroenteritis with protein-losing enteropathy are not common. To our knowledge, no case has reported in Korea. So we report this case with brief review of the literature.
Abdominal Pain ; Adult ; Ankle ; Diarrhea ; Edema ; Electrolytes ; Eosinophilia ; Eosinophils* ; Gastroenteritis* ; Gastrointestinal Tract ; Humans ; Hypoalbuminemia ; Korea ; Male ; Protein-Losing Enteropathies ; Thigh

BACKGROUND: Arbutin is a glycosylated hydroquinone found at high concentration in certain plants capable of surviving extreme and sustained dehydration. It has been suggested as an inhibitory compound of melanogenesis. OBJECTIVE: The purpose of this study was to investigate the effect of arbutin on melanogenesis in cultured human melanocytes and to evaluate the effectiveness of arbutin in patients with melasma. METHODS: I. In vitro study:we examine the cell number, SRB assay, tyrosinase activity, and melanin contents of cultured human melanocytes in control(absence of arbutin) and experimental groups (presence of 10(-5) M, 10(-4) M, and 10(-3) M arbutin). II. In vivo study:6 patients with melasma applied a 3 % arbutin solution twice daily for 8 weeks. Clinical response to treatment was evaluated by patients' subjective assessment and MASI(Melasma Area and Severity Index) score after 8 weeks of treatment. RESULTS: I. In vitro study 1. The number of melanocytes was decreased in groups treated with 10(-5) M, 10(-4) M, 10(-3) M arbutin for 2 days and 10(-4) M, 10(-3) M arbutin for 7 days. 2. On SRB assay, the proliferation of melanocytes was decreased in groups treated with 10(-5) M, 10(-4) M, 10(-3) M arbutin for 2 days and for 7 days. 3. Tyrosinase activity was decreased in groups treated wtih 10(-4) M, 10(-3) M arbutin for 2 days and 10(-5) M, 10(-4) M, 10(-3) M arbutin for 7 days. 4. The melanin contents were decreased in group treated with 10(-3) M arbutin for 7 days. II. In vivo study 1. On patients'subjective assessment, one showed moderate improvement, one showed mild improvement, and the other four showed no change. 2. On MASI score, there was less than 10% improvement in all 6 patients. 3. Side effects were not found in all 6 patients. CONCLUSION: Although arbutin showed an inhibitory effect on cell proliferation, tyrosinase activity, and melanin synthesis in cultured human melanocytes, there was no significant effect of depigmentation in the patients with melasma.
Arbutin* ; Cell Count ; Cell Proliferation ; Dehydration ; Humans ; Melanins ; Melanocytes ; Melanosis ; Monophenol Monooxygenase

A case of generalized pustular psoriasis in a 12 year-old girl is described. She had generalized pustular eruptions on erythematous base, presenting lake of pus, yellowish brown crusts and denudation associated with high fever and chills. With the administration of oral etretinate at a dose of 0.8mg/kg/day for 1 week, rapid and dramatic remission was achieved. About 3 months after her discharge, there developed another wave of new pustules with fever. She was again placed on etretinate therapy. However, there was no expected response as before with developing new pustules continuously. At that time, we tried combination therapy with etretinate and methotrexate. the pustules and erythema began to remit, with eventual clearing of the skin in 6 weeks.
Acitretin* ; Child ; Chills ; Erythema ; Etretinate* ; Female ; Fever ; Humans ; Lakes ; Methotrexate* ; Psoriasis ; Skin ; Suppuration

No abstract available.
Cartilage* ; Follow-Up Studies*

Reports of systemic candidiasis in neonates have been noted with major advances in neonatal care permitting the frequent survival of low birth weight infants. We experienced 2 cases of systemic candidiasis associated with skin involvement in very low birth weight infants. One case presented with abdominal distension and an erythematous maculopapular eruption on trunk, buttock, and lower extremities. Culture of peritoneal fluid grew Candida of albicans. In another case, a diffuse erythematous patch was noted on the back and buttock. Culture of blood sample grew Candida of albicans. Despite the treatment with intravenous fluconazole, clinical status of the patients continued to remain unstable, and they died.
Ascitic Fluid ; Buttocks ; Candida ; Candidiasis* ; Fluconazole ; Humans ; Infant* ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Lower Extremity ; Skin

No abstract available.
Laser Therapy*