Epidermolytic keratosis palmaris is a rare disease which shows clinical findings of Unna Thost keratoderma and histopathologic of epidermolytic hyperkeratosis. We report herein a case of epidermolytic keratosis palmaris et plantaris in a 16-month-old female baby. Light microscopy shows marked hyperkeratosis, large irregular keratohyalin granules, and large clear spaces in the granular and upper spinous layers. Eletron microscopic findings shows that the clear spaces are areas of cytoplasm filled with a fibrillar material and cellular organelles. Abnormal clumping of tonofilament and keratohyalin is also present.
Cytoplasm ; Female ; Humans ; Hyperkeratosis, Epidermolytic ; Infant ; Intermediate Filaments ; Keratoderma, Palmoplantar* ; Keratosis* ; Microscopy ; Organelles ; Rare Diseases

Multiple dermatofibromas are rare and some case reports show this disease has been related to generalized defective immune function: autoimmune disease and altered immunity, such as AIDS and systemic lupus erythematosus(SLE), can be treated with immunosuppressive agents. But there are no specific reports of this disease developing in healthy people. We describe a case of multiple dermatofibromas in a 38-year-old healthy man who showed no abnormality of the immune function.
Adult* ; Autoimmune Diseases ; Histiocytoma, Benign Fibrous* ; Humans ; Immunosuppressive Agents

Xanthogranulomatous pyelonephritis (XGP) is a rare, severe, chronic infection of the kidney resulting in the normal renal parenchyma being replaced by characteristic lipid-laden macrophages (foam cells). It may occur at any age but is extremely rare in children. Correct pre-operative diagnosis is essential for appropriate surgical approach. We report a case of diffuse XGP in a 6-year-old boy which was pre-operatively diagnosed as XGP.
Child* ; Diagnosis ; Humans ; Kidney ; Macrophages ; Male ; Pyelonephritis, Xanthogranulomatous*

Arteriovenous malformation (AVM) of the scrotum is a rare vascular lesion, which is distinguished histologically by ambiguous vessels displaying both arterial and venous characteristics and abnormal muscularization of variable sized ectatic vessels. Complete excision is recommended as a definitive treatment. Here, a case of arteriovenous malformation involving the scrotal vessels is reported.
Arteriovenous Malformations* ; Scrotum

PURPOSE: The goal of intermittent androgen deprivation (IAD) therapy in prostate cancer patients is to delay the disease progression and improve the survival rate. Therefore, the duration of off-treatment is very important for the effective treatment outcome of IAD. We analyzed factors that influence the duration of off-treatment in IAD. MATERIALS AND METHODS: We reviewed the medical records of 45 patients with prostate cancer who had completed at least 1 cycle of IAD. Uni- and multi-variate tests were used to determine the factors, which are predictive to the duration of off-treatment. These factors included: the patient's age, biopsy Gleasons score, initial PSA, presence of bone metastasis, PSA levels at 3 months following on-treatment and at 3 months following off-treatment, and the duration of on-treatment. RESULTS: The average follow up duration was 34 months (15-71 months). The average off-treatment duration of each cycle was 11.1 (4-40), 7.5 (4-14), and 5.6 (3-10) months for the 1st, 2nd and 3rd cycles, respectively. Independent factors associated with the extension of duration of off-treatment, by univariate tests, included: initial PSA value, PSA values at 3 months following on-treatment, PSA at 3 months following off- treatment, and duration of on-treatment. The duration of off-treatment was inversely related to the serum PSA level at the start, 3 months following on-treatment, and 3 months following off-treatment, while it was directly related to the duration of on- treatment by multivariate tests. CONCLUSIONS: The pretreatment serum PSA level and the serum PSA level at 3 months following on-treatment and off-treatment were valuable predictors for the duration of off-treatment in IAD.
Biopsy ; Disease Progression ; Follow-Up Studies ; Humans ; Medical Records ; Neoplasm Metastasis ; Prostate* ; Prostate-Specific Antigen ; Prostatic Neoplasms* ; Survival Rate ; Treatment Outcome

PURPOSE: This paper evaluate and classified the risk factors that could possibly cause ischemic stroke in children, and investigate whether TOAST classification could be applied to the ischemic stroke in children. METHODS: From March 1995 to February 2002, we retrospectively reviewed the medical record of 87 patients under the age 16 who had been registered to the Ajou Stroke Registry. We evaluated the risk factors of ischemic stroke according to the Ajou Stroke Registry Protocol, and classified the risk factors into 6 main subgroups. In addition, the risk factors were investigated according to the age distribution. TOAST classification had been applied to children with ischemic stroke. RESULTS: There was 64 ischemic stroke(73.6%) and 23 hemorrhagic stroke(26.4%) among the 87 patients. In children with ischemic stroke, there was three major peak age; 13 patients younger than 1 year old(15.3%), 26 patients in age 5 to 8 years old(40.6 %), 22 patients in 9 to 12 years old(34.3%). Vasculopathy(48.6%) was the most common risk factor of the ischemic stroke in this study. Other important risk factors in order of frequency were hypertensive encephalopathy(12.5%), infectious disease(7.8%), metabolic disease(7.8%), and hematologic disease(6.2%). Moyamoya disease was the most common cause of ischemic stroke due to vasculopathy. If we analyze the risk factors according to the age at the presentation of ischemic stroke, 90% of the ischemic stroke due to vasculopathy had been developed after 5 years of age. However, ischemic stroke due to infectious disease had been developed less than 4 years of age. Major stroke was the most common subtype of the ischemic stroke in children, but TIA had been found in 80% of the patients with ischemic stroke due to moyamoya disease. TOAST classification determined the subtype of the ischemic stroke only in 2 children. Risk factors of ischemic stroke in the remained 62 patients were extremely variable, and could not be applicable to the TOAST classification. CONCLUSION: Risk factor of ischemic stroke were found in the 90% of patients. Although risk factors of ischemic stroke were extremely variable in children, cerebral vasculopathy including moyamoya disease was the most common. However, inherited metabolic disorder, coagulopathy, and hypertensive encephalopathy were also relatively common risk factors of ischemic stroke especially in children older than 5 years old. Therefore, we must extensively evaluate all the possible risk factors of ischemic stroke in children. TOAST classification could not be applicable in children because the risk factors of ischemic stroke were extremely variable.
Age Distribution ; Child* ; Child, Preschool ; Classification ; Communicable Diseases ; Humans ; Hypertensive Encephalopathy ; Medical Records ; Moyamoya Disease ; Retrospective Studies ; Risk Factors* ; Stroke*

No abstract available.
Niemann-Pick Diseases*

The subconjuctival sparganosis is rare tissue helminthiasis which develops frequently in. orbital, abdominal, urethral, ureteral and vertebral cases. Authors reported a case of sparganosis which complained conjuntival hyperemia, chemosis, subconjuntival mass in the right eye for 10 months. The patient was a 37 years old male who had a history of having eaten raw snake in the So-Re Gun. Gyeonggi Do for the purpose of tonics 3 years ago. In the winter of 1977, he had experienced edematous changes in the right cheek intermittently. A few months later, he noticed the apperence of the congestion and a mass on the right eye. On surgical exploration, one larvae (5.8 cm in length and 5 m min width) was extracted from the right upper temporal subconjunctiva. It was actively motile in the bottle filled with saline, so this larvae was consulted to parasitology department of Korea University from our clinic under the impression of subconjunctival sparganosis. The sources of infection of some formely reported sparganosis in Korea were discussed. It is most probable that human sparganosis is acquired by consuming of raw snakes and frogs in this country.
Adult ; Cheek ; Estrogens, Conjugated (USP) ; Gyeonggi-do ; Helminthiasis ; Humans ; Hyperemia ; Korea ; Larva ; Male ; Orbit ; Parasitology ; Snakes ; Sparganosis* ; Ureter

Sudoriparous angioma is an unusual form of angioma with pain and hyperhidrosis. We present two cases of typical sudoriparous angioma. Case 1: 9-year-old female showed 0. 8 to 2. 0cm multiple dark brownish tender palpable hypertrichotic masses on the right lower leg after birth. Muscular atrophy and flexion contracture was also developed on the right lower leg for 2 years. Case 2: 4-year-old rnale showed 3 * 3cm tender elevated mass on the left shin after birth. Marked hyperhidrosis was developed when even slight pressure applied on the lesion in these cases. On histologic examination, these two cases showed proliferation of varible sized blood veasels hyperplasia and dilated hyperplastic eccrine glandular and ectopic adipose tissue on the mid and deep dermis. Muscular biopsy from case 1 showed fibrotic and degenerative change. Electromicroscopic examination done from case 1. The secretory epithelial cell of eccrine gland showed cytoplastic degradation and slight dilated intercellular canaliculi (Fig. 11). The lumen of subepithelial capillaries were dilated and some of endothelial cells showed degenerative changes evidenced by RER dilation, mitochondrial swelling and detachment from underlying tissue(Fig. l2). The cutaneous nerve showed many non-myelinated axons enclosed in cytoplasm of Schwann cells.
Adipose Tissue ; Axons ; Biopsy ; Capillaries ; Child ; Child, Preschool ; Contracture ; Cytoplasm ; Dermis ; Eccrine Glands ; Endothelial Cells ; Epithelial Cells ; Female ; Hemangioma* ; Humans ; Hyperhidrosis ; Hyperplasia ; Leg ; Mitochondrial Swelling ; Muscular Atrophy ; Parturition ; Schwann Cells

Leiomyoma Cutis is an unusual benign tumor of smooth muscle derivecl either from the arrector pili muscle, from the media of blood vesseles, or from smooth muscles of scrotum, labia or nipple. Two casea of leiomyoma cutis confirmed by H&E stain and Massons stain is presented, one with lesion on the left thigh and the ather with lesion of the left ankle. The first case was a 49 year-old female with solitary pinhead sized subcutaneous firm mass with intermittent pain and tenderess for 10 months, The second case waa a 36 year-old female with painful solitary peanut sized firm nodule which had pinkish center of 1 year duration.
Adult ; Ankle ; Blood Vessels ; Female ; Humans ; Leiomyoma* ; Middle Aged ; Muscle, Smooth ; Nipples ; Scrotum ; Thigh