• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.606-609
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• 2003

Goltz syndrome(focal dermal hypoplasia) is a rare disorder characterized by ectodermal and mesodermal dysplasia described in 1962 by Goltz. In Korea, one case of Goltz syndrome was reported in 1994. The inheritance mode is mostly X-linked dominant. Skin abnormality is the most common manifestation including hypoplasia of the dermis. Skeletal involvement such as syndactyly, polydactyly, scoliosis, kyphosis and spina bifida occulta may be present, also ocular and dental abnormalities are reported. Radiologic findings are the osteopathy and striation of the long bone. We experienced a case of Goltz syndrome in a 9-year old female who was presented with right side hypotrophy, focal dermal hypoplasia, ocular(anidria, microcornea), dental(oligodontia, amelogenesis) and skeletal(syndactyly) abnormalities. Skin biopsy was performed and showed decreased expression of type I collagen gene with Northern blotting.

• Archives of Craniofacial Surgery
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• v.9 no.1
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• pp.31-34
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• 2008

Introduction: Lymphoepithelioma-like carcinoma of the skin (LELCS) is a rare cutaneous tumor of low grade malignancy and microscopically resembles lymphoepitheliomatous malignancies in the nasopharynx, palatine tonsils, salivary glands and uterine cervix. LELCS presents as scarlet-colored firm nodules or plaques on the face, scalp, or shoulder of middle-aged to elderly individuals. Material and Methods: A 72 year-old female had complained a papule like lesion with intermittent pruritis on the left temple for 2 years. But the lesion was changed to scarlet-colored firm nodule with ulceration. The mass was diagnosed as LELCS on the biopsy. And to confirm that the mass is not metastatic lymphoepithelioma-like carcinoma from other sites or direct tumor extension from the nasopharynx, selective radiographic and laboratory tests were done carefully. Results: Wide excision was performed with general endotracheal anesthesia. The tumor is composed of island of large epithelial cells surrounded by as dense infiltrate of lymphocytes. Immunohistochemical staining with cytokeratin and epithelial membrane antigen(EMA), the tumor cells were positive reaction for stain. And Epstein-Barr virus genome was not detected by in situ hybridization. So, the tumor was confirmed as LELCS. Conclusion: LELCS was described by Swanson at 1988, but has not been reported in the field of plastic surgery of Korea. We report a case of primary LELCS that occurred in the left temple with clinical characteristics, histologic features and references.

• Archives of Plastic Surgery
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• v.38 no.1
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• pp.89-92
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• 2011

Purpose: Electrical burn of scalp is uncommon. Much more, chronically exposed dura in unstable burn scar is quite exceptional. Hence, we report a case of chronically exposed dura following electrical burn. Methods: A 63-year-old man presented with an about 40 years history of an ulcerative lesion arising from electrical burn scar with 'squeeze like sensation' around wound. Wound was about $6{\times}8$ cm. Area in the center was $3{\times}3$ cm nonviable dura without sequestrum. Tangential excision with an intraoperative neurosurgical consultation and transposition flap under general anesthesia was done. Intraoperative biopsy was done. The wound was diagnosed as chronic osteomyelitis, not Marjolin ulcer. Flap was taken successfully. But after 5 days, infectious discharge had been appeared during 2 weeks, despite irrigation and drainage. As flap was re-evaluated, we could see remnant necrotic dura. After that, latissimus dorsi muscle free flap with meshed split thickness skin graft was transferred without excision of necrotic dura. Results: Flap was taken successfully. Follow-up at 10 weeks has been uneventful, with good and stable coverage of the wound. Conclusion: It is true that complete excision of devitalized tissue with sagittal sinus obliteration is prerequisite to flap taken. But necrotic dura was tangentially excised instead of total dura excision, because, posterior two-thirds of the sagittal sinus was involved underneath. Muscle is rich in blood vessels and decrease the recipient-site bacterial count effectively. In this case, muscle flap with skin graft without total dura excision is an alternative treatment.

• Archives of Plastic Surgery
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• v.35 no.6
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• pp.743-747
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• 2008

Purpose: Malignant changes of Marjolin's ulcer arising from chronic burn scar are rare. The majority of them are squamous cell carcinoma and basal cell carcinoma. Malignant fibrous histiocytoma is a deep seated pleomorphic sarcoma, which occurs principally as a mass of the extremities, abdominal cavity, or retroperitoneum in adults. Methods: We report a 58-year-old male patient who was admitted due to $3.5{\times}5cm$ chronic ulceration of anterior chest wall on the center of old burn scar. His scar had been occurred by boiling oil and treated with conservative treatment 45 years ago. Preoperative punch biopsy showed suspicious malignant changes and contrast enhanced chest CT showed well-defined, irregular shape enhancing lesion on anterior chest wall without intrathoracic metastasis. Results: The tumor was widely excised and defect was covered with skin graft without infection, necrosis and any other complication. The pathologic findings are compatible with malignant fibrous histiocytoma(storiform - pleomorphic type). The patient underwent 3 cycles of chemotheraphy. Although distant metastasis to the lung developed 6 months later and the patient died 9 month later, there was no local reoccurrence. Conclusion: Aggressive and early excision is needed because malignant fibrous histiocytoma has characteristics of high malignancy with a propensity for early and distant spread. Furthermore, the patient's education about disease entity and postoperative regular follow-up for local recurrence or metastasis is very important. To prevent malignancy from secondly healing burn scar, early skin graft is recommended for patients with deep second degree burn.

• Archives of Plastic Surgery
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• v.33 no.1
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• pp.13-20
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• 2006

Alginate, which is isolated from brown seaweed, is a bioabsorbable long chain polysaccharides, ${\beta}$-D-mannuronic acid and ${\alpha}$-L-guluronic acid. The authors produced alginates and alginate-colllagen as a disc form. Then, to evaluate the efficacy of alginate and alginate-collagen complex as a wound healing material, three full-thickness skin defects of 2 cm in diameter were made at the back of the New Zealand white rabbits. Three groups of dressing materials-alginate, alginate-collagen complex and vaseline gauze as control group - were applied on the wound and the results were evaluated grossly and histopathologically. The authors compared gross findings of sizes of healed wound, wound epithelization and wound contraction by tracing the remaining wound area at 5th, 10th, 15th, 20th, 25th day after wound introduction, and wound biopsy was performed at 3rd, 7th, 14th, 21st day, respectively. Alginate and alginate-collagen complex showed statistically higher percentage of wound contraction and wound healing compared to control group(p<0.05). Alginate-collagen complex showed statistically higher percentage of wound contraction, epithelization and wound healing compared to alginate alone. In conclusion, the result suggests that alginate has a good effect of wound healing and that alginate-collagen complex is more effective in wound healing than alginate alone.

• Journal of Oral Medicine and Pain
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• v.38 no.2
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• pp.103-108
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• 2013

Pachyonychia congenita is a very rare group of an autosomal dominant genodermatosis caused by heterozygous mutations in the keratin genes. The clinical findings affect nail and toenails, soles, and oral mucosa, etc. The main symptoms include nail and toenail dystrophy, hyperkeratosis of hands and feet, follicular hyperkeratosis, oral leukokeratosis. Many therapeutic modalities have been used to treat skin lesion, including surgical and mechanical procedures, chemical agents, medications. Oral lesions but not usually require treatment, if the patient's discomfort occurs, symptomatic therapy is performed. In the patients accompanied by oral and skin lesions, clinician have to observe specific manifestations with dystrophy of the fingernails and toenails, plantar hyperkeratosis, oral leukokeratosis and tissue biopsy is required for diagnosis confirmed.

• Korean Journal of Veterinary Research
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• v.46 no.2
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• pp.165-169
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• 2006

A 6-year-old female Jindo dog weighing 20 kg was presented to the Veterinary Medical Teaching Hospital of Seoul National University for the formation of crust and erosion of the nasal bridge and planum, and for alopecia of ear margin. Erythema, crusts, alopecia and erosions were present in the nasal planum and the bridge of the nose on physical examination. There were no abnormalities on CBC, serum chemistry, and basic dermatologic examination which included skin scraping, bacterial cultures, and fungal cultures. On cytology, the acantholytic cells were surrounded by non-degenerate neutrophils. Biopsy samples obtained from skin lesionswere diagnosedpemphigus erythematosus by histopathology and immunochemical staining. Therefore the dog was initially treated with oral prednisolone as an immunosuppressive dose for tour weeks, but clinical manifestations did not improve so far.

• Tuberculosis and Respiratory Diseases
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• v.46 no.3
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• pp.409-413
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• 1999

Malignant melanoma develops from the melanocyte and the most common primary site is skin, followed by mucosa and retina. Even though any other tissue where melanocytes reside could be the primary site of the malignant melanoma, the one developed in the mediastinum is rarely reported. We experienced a patients of 54 years old woman whose initial symptom was progressive dyspnea for one month, and proved to have the anterior mediastinal mass with pleural effusion and the small mass in the abdominal soft tissue. The needle aspiration biopsy from the mediastinal mass showed the consistent findings with malignant melanoma. We concluded the mediastinum was the primary site of the malignant melanoma of this patient because we couldn't find any other evidence of primary tumor in skin, oral and gastrointestinal mucosa, and retina. She has been treated with combined chemotherapy with dacarbazine, cisplatin and vinblastine. Her symptom was improved after chemotherapy and follow up chest CT after three cycles of chemotherapy showed the decreased tumor size in the mediastinum.

• Archives of Plastic Surgery
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• v.38 no.5
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• pp.711-714
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• 2011

Purpose: Angiosarcoma is a rare malignant neoplasm of endothelial type cells that line vessel walls. It tends to occur in aged male and the prognosis of angiosarcoma is very poor because of frequent local recurrence and early metastasis. The treatment regimen is yet to be established from its rare occurrence but the wide excision in early stage is known to be the most effective. The authors report two cases of near totally excised angiosarcoma with more than a safety margin of 5 cm. Methods: The two subjects were aged male patients, one of the two was diagnosed with angiosarcoma from our institution confirmed by the biopsy. The other one went through the wide excision with a safety margin of 2 cm and split-thickness skin graft but local recurrence was observed. The two patients underwent near total excision with more than a safety margin of 5 cm, leaving only the periosteum. After confirming that the angiosarcoma had not infiltrated the excision margin, reconstruction with split-thickness skin graft was performed. Results: Based on 6 months and 24 months post-surgery assessment, no local recurrence or remote metastasis in the lungs, liver, bones, and lymph nodes at the neck, where remote metastasis is common, was reported by the two subjects who underwent near total excision with a safety margin of 5 cm. Conclusion: Angiosarcoma has very poor prognosis from its frequent recurrence and metastasis. To enhance the survival rate of angiosarcoma patients, early diagnosis, timely surgical treatment, and radiotherapy after surgery are critical. In addition, authors suggest that it is necessary to further study the efficacy of wide excision using a wider safety margin as much as possible, and to apply this to more cases.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.2
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• pp.233-239
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• 1999

Acrodermatitis enteropethica (AE) is a rare autosomal recessive disorder of zinc absorption leading to chronic diarrhea and characteristic skin lesion. The term is also applied to any acquired zinc deficiency state resulting in the same clinical pictures. We experienced one case of AE in 1 month old male infant who had bacterial enterocolitis. The skin around mouth, anus, eyes, ears, hands and legs became reddish, vesicular and eczematoid. Serum zinc level was decreased to $51.4\;{\mu}g/dL$ (N=70~150). Endoscopic finding revealed pale gastric mucosa and villous atrophy of small intestine. Biopsy finding of small intestine showed no villi due to mucosal atrophy. On 13 day of admission jaundice with DIC were noted and AST & ALT were elevated to 110 & 36.8 IU/L, respectively. Diarrhea was improved but jaundice and liver function were not recovered until discharge from hospital. After discharge when the patient was 4 months of age serum bilirubin and AST/ALT had not been normalized. CMV shell vial culture of urine and CMV Ig G antibody were positive. So intravenous ganciclovir injection of 7.5 mg/kg, two times a day for 2 weeks and then 10 mg/kg/day for 3 months was done from 4 to 6 months of age. No virus was found in the urine and AST & ALT were normalized at 2 months after stopping ganciclovir treatment.