Congenital ichthyosiform erythroderma is most severe type of ichthyosis. This disease is characterized clinically by generallized erythroderma covered with flaccid bullae and verruciform scales which are especially prominent on the flexural areas. This disease demonstrates an autosomal dominant mode of inheritance. The distinctive histologic change is the extreme hyperkeratosis associated with granular and vacuolar degeneration of the mid and upper malphigian layers. Cell kinetic studies indicate an increase in the epidermal mitotic turnover tirne. This 8 months old male patient visited to Dermatologic Department of Severance Hospital on April-28, 1973. Seven days after birth the scales were appeared on his flexural region of limbs and shed to leave a raw surface which forms scales anew. And there were the intermittent appearance of flaccid bullae prominantly involve the flexural area of limb but thereafter appearing the other region of body. Skin biopsy shows that of bullous type of congenital ichthyosiform erythroderma. This baby was expired 4 days after the admission day in spite of vigorous systemic and local trea- tments. The autopsy findings had exhibited fatty necrotic degenerative changes of hepatic cells and septic inflammatory changes of meningeal vessels and meninges itself as the causes of death of this pitiful victim.
Autopsy ; Biopsy ; Cause of Death ; Dermatitis, Exfoliative ; Extremities ; Hepatocytes ; Humans ; Ichthyosiform Erythroderma, Congenital ; Ichthyosis ; Infant ; Male ; Meninges ; Parturition ; Skin ; Weights and Measures ; Wills

Lymphangioma circumscriptum is a rare disease and the lesions are indistinguishable, clinically and histologically, from lymphangiectasis. The only difference is that lymphangiectasis is usually bilateral and develops in adult or late life, ie, condary to irradiation, surgical interruption, or some malignancies, but lymphangioma circumscriptum is congenital disease or develops soon after birth.
Adult ; Humans ; Lymphangiectasis ; Lymphangioma* ; Parturition ; Rare Diseases

No abstract available.
Mucocutaneous Lymph Node Syndrome*

We have experienced a patient (4-year-old female) with a granular cell tumor (GCT), Examination of patients skin showed a bean sized asymtomatic firm nodule on the left baek. Exciaional biopsy and histopathologic examination were perforrned under the impression of angiolipoma. The specirnen revealed a distinctive picture of GCT.
Angiolipoma ; Biopsy ; Granular Cell Tumor* ; Humans ; Skin

No abstract available.
Porphyria, Acute Intermittent* ; Pregnancy*

BACKGROUND AND OBJECTIVES: Little information about the surgical anatomy and technique for retropharyngeal node dissection has been published. The purpose of this study was to review our surgical technique and results of retropharyngeal lymph node dissection. MATERIALS AND METHODS: Eleven advanced oropharyngeal and hypopharyngeal squamous cell carcinoma patients who had been treated with resection of primary tumor and standard neck dissection including retropharyngeal lymph node dissection from 1994 to 1999 were evaluated retrospectively. RESULTS: One of 11 patients had positive retropharyngeal lymph node. The surgical technique used for retropharyngeal lymph node dissection were total laryngopharyngectomy, mandibular splitting or mandibulectomy approach. There was no specific complication of retropharyngeal lymph node dissection except one case of Horner's syndrome. CONCLUSION: Retropharyngeal lymph node dissection was a safe procedure, bet it required total laryngopharyngectomy, madibular splitting or mandibulectomy approach. It was possible to remove retropharyngeal lymph made en-bloc with primary tumor in most cases.
Carcinoma, Squamous Cell ; Horner Syndrome ; Humans ; Hypopharyngeal Neoplasms ; Lymph Node Excision* ; Lymph Nodes* ; Neck Dissection ; Oropharyngeal Neoplasms ; Retrospective Studies

Cutaneous larva migrans(creeping eruption) is a rare cutaneous serpiginous erution caused by the accidental invasion of the skin by parasites. We report herein a 27 year-old female with a mild pruritic erythematous serpiginous linear skin lesion located on the patient's back for a month. Histopathologic examination revealed a larva in the dermis with dense iinfiltrates of eosinophils and lymphocytes. After treatment with oral anitiparasitics, the skin lesions resolved with post-inflammatory hyperpigmentation. This is the first case of larva migrans showing the larva on the biopsy specimen in Korea.
Adult ; Biopsy* ; Dermis ; Eosinophils ; Female ; Humans ; Hyperpigmentation ; Korea ; Larva Migrans* ; Larva* ; Lymphocytes ; Parasites ; Skin

Serum IgE levels in 48 patients with scabies were measured by enzyme-linked immunosorbent a.ssay(ELISA) to evaluate the relationship between the scabies and serum IgE. A group of 44 healthy persons was taken as a controls. The results were summarized as follows: In children group(p 15 year-old) of the scabies, serum IgE level was 446 3 +267.2 IU/ml and significantly elevated than in controls, 126, 3 175, 4 IU/rnl, In adult group(over 16 year-old), serum IgE levels of the scabies and controls were 506. 4 + 581. 8 IU/ml, 435, 5+ 548, 8 lU/rnl respetively, and there was no significant difference between them. 2) There were no statistical correlations between the serum IgE levels and intensity of pruritus, duration of illness, number of skin lesions or number of circu1ating eosinophils in patients with scabies.
Adult ; Child ; Eosinophils ; Humans ; Immunoglobulin E* ; Pruritus ; Scabies* ; Skin

Radioimmunoassay for the measurenment of plasma renin activity (PRA) was performed in 43 normal Koreans and 67 patients with essential hypertension. Urinary sodium excretion rates were measured in the concurrently collected 24 hour urine samples as an index of their sodium balance. The results were as follows: 1. There was an inverse correlation between 24 hour sodium excretion and PRA. The normal values of PRA in supine position ranged from 1.0 to 7.0 ng/ml/hr. When 24 hour sodium excretion were between 50 to 150 mEq. PRA in elderly persons tended to be low. 2. Of the 67 patients with essential hypertension, PRA was low in 20 cases (29.9%), normal in 36 (53.7%), and high in 11 cases (16.4%). 3. In the normal and high renin groups, who tended to be younger in ages, blood urea nitrogen was slightly higher than in low renin group, and hypertensive retinopathy was more prevalent in normal and high renin groups. 4. There were 17 cases with cardiovascular complications(12 with cerebrovascular accidents, 5 with heart failure): 6 in low renin group (30%), 9 in normal (25%) and 2 in high renin group (18.2%). There was no evidence that more frequent complications developed in higher renin groups.
Aged ; Blood Urea Nitrogen ; Heart ; Humans ; Hypertension* ; Hypertensive Retinopathy ; Plasma* ; Radioimmunoassay ; Reference Values ; Renin* ; Sodium ; Stroke ; Supine Position

PURPOSE: The treatment of a clinically node-positive (cN+) neck is important in the management of oral cavity squamous cell carcinoma (OSCC). However, the extent of neck dissection (ND) remains controversial. The purpose of our study was to evaluate whether level IV or V can be excluded in therapeutic ND for cN+ OSCC patients. MATERIALS AND METHODS: We performed a retrospective chart review of 92 patients who underwent a comprehensive or selective ND as a therapeutic treatment of cN+ OSCC from January 1993 to February 2009. RESULTS: The incidence rate of metastasis to level IV or V was 22% (16 of 72) on the ipsilateral neck. Of 67 cases without clinically suspicious nodes at level IV or V, 11 cases (16%, 11 of 67) had pathologically proven lymphatic metastasis to level IV or V. Only a nodal staging above N2b was significantly relevant with the higher rate of level IV or V lymph node metastasis (p=0.025). In this series, selective ND, combined with proper adjuvant therapy, achieved regional control and survival rates comparable to comprehensive ND in patients under the N stage of cN2a OSCC. CONCLUSION: In conclusion, level IV and V patients can avoid recurrence under cN2a OSCC.
Adult ; Aged ; Carcinoma, Squamous Cell/mortality/*pathology/*surgery ; Chemoradiotherapy ; Disease-Free Survival ; Female ; Humans ; Lymphatic Metastasis ; Male ; Middle Aged ; Mouth Neoplasms/mortality/*pathology/*surgery ; Neck/surgery ; *Neck Dissection ; Neoplasm Metastasis ; Radiotherapy, Adjuvant ; Retrospective Studies ; Treatment Outcome