Purpura fulminans may be seen in three different clinical settings: (1) in the neonatal period from protein C and S deficiencies, (2) during severe bacterial infections such as “sepsis-associated” purpura fulminans, and (3) during the convalescence of an otherwise benign “preparatory” infectious disease most commonly involving the skin. We report a case of a 20-month-old male child with purpura fulminans as a presenting sign of disseminated intravascular coagulopathy (DIC). He had suffered from fever of unknown origin for a month. Although purpura fulminans is not a common disorder to dermatologists, the awareness of this disorder may be the clue to diagnose and treat underlying diseases.
Bacterial Infections ; Child ; Communicable Diseases ; Convalescence ; Fever of Unknown Origin ; Humans ; Infant ; Male ; Protein C ; Purpura Fulminans* ; Purpura* ; Skin

BACKGROUND: Mixed tumor of the skin or chondroid syringoma is a benign neoplasm characterized by histological features of a mixture of epithelial and mesenchymal components. It is a rare and benign appendageal tumor, found mostly on the head and neck, and present as an asymptomatic, firm, subcutaneous nodule. There have only been a few reported cases in Korea. OBJECTIVES: This study was aimed to characterize the clinical and histopathlological features of mixed tumor of the skin. METHODS: We reviewed the clinical data and histologic slides of seven patients who have been diagnosed with mixed tumor of the skin by histopathological examination. RESULTS: There were six male patients and only one female. Age of onset of mixed tumor of the skin varied from 26 to 65 years. A11 patients had the lesion on the head: perioral area (3 cases), nose (2 cases), cheek (1 case), and temple area (1 case). Each tumor was a solitary, asymptomatic, and firm, about 0.5-1.5 cm sized, subcutaneous nodule. Histopathologically, all 7 cases presented apocrine differentiation. Two cases showed follicular differentiation, and 2 cases showed sebaceous differentiation. Every tumor showed myxoid stroma except two with typical chondroid matrix. Adipose metaplasia of the matrix was present in 2 cases. In 3 cases, the so-called hyaline cells were rich in the stroma. CONCLUSION: Mixed tumor of the skin was most commonly seen as an asymptomatic, firm subcutaneous nodule on the head. Tumors showing apocrine differentiation were more common than that of eccrine differentiation, All 7 cases presented apocrine differentiation. Follicular and sebaceous differentiation might occur in apocrine type of mixed tumors of the skin. The stroma of mixed tumor of the skin might be myxoid, chondroid, or adipose.
Adenoma, Pleomorphic ; Age of Onset ; Cheek ; Female ; Head ; Humans ; Hyalin ; Korea ; Male ; Metaplasia ; Neck ; Nose ; Skin*

BACKGROUND: Currently the most common treatment modality of refractory ascites in patients with liver cirrhosis was large volume paracentesis, but this procedure usually needed albumin infusion and occasionally developed unwanted complications. By reason of albumin shortage in Korea and occasional unfavorable complications, we studied the usefulness of dialytic ultrafiltration as an another treatment modality of refractory ascites. METHODS: Dialytic ultrafiltration was done in 10 patients (total 48 times) with liver cirrhosis or hepatocellular carcinoma. Two drainage conduit (via 16 gauge angio-catheter) of input and output were made by puncture of patient's right and left lower quadrant abdomen. The initial ultrafiltration rate of dialyser was 250mL/min. Ascitic fluid was removed continuously until the filtration rate down at 50mL/min. After ultrafiltration, ascitic fluid contained concentrated albumin and large molecules was reinfused via input conduit. Pre-treatment and post-treatment level of blood chemistry, plasma renin concentration, aldosterone, and electrolytes in serum; total protein and albumin in ascites were measured. During the ultrafiltration, we closely observed the change of blood pressure, heart rates and mental status. RESULTS: The mean ultrafiltration time was 231+/-28min, ultrafiltrated volume was 5.15+/-1.41 L. During dialytic ultrafiltration, patient's blood pressure and heart rate were stable and there was no change of mental status. After dialytic ultrafiltration, blood urea nitrogen level significantly decreased from 30.5+/-23.7mg/dL to 25.7+/-20.2mg/dL; serum aldosterone level decreased from 807.3+/-301.1pg/ml to 431.1+/-187.2pg/ml in serum (P<0.01). The albumin level in the ascitic fluid significantly increased from 0.67+/-0.28g/dL to 1.90+/-1.16g/dL (P<0.01). Plasma renin concentration level tend to decreased (P=0.06). The patient's serum total protein, albumin, electrolytes, and creatinine were not changed. Complications of dialytic ultrafiltration were peritonitis (one case) and hypotension (one case). But these unwanted complications were readily managed by adequate antibiotics and intravenous fluid therapy. CONCLUSION: The dialytic ultrafiltration can be used effectively without albumin infusion in the treatment of refrartory ascites in patients with advanced liver cirrhosis.
Abdomen ; Aldosterone ; Anti-Bacterial Agents ; Ascites* ; Ascitic Fluid ; Blood Pressure ; Blood Urea Nitrogen ; Carcinoma, Hepatocellular ; Chemistry ; Creatinine ; Drainage ; Electrolytes ; Filtration ; Fluid Therapy ; Heart Rate ; Humans ; Hypotension ; Korea ; Liver Cirrhosis* ; Liver* ; Paracentesis ; Patient Rights ; Peritonitis ; Plasma ; Punctures ; Renin ; Ultrafiltration*

Idiopathic mesenteric phlebosclerosis, rare disease entity causing chronic mesenteric ischemia is a member of non-thrombotic, non-inflammatory stenosis or occlusion of the mesenteric veins. The histologic hallmark is marked fibrous mural thickening and sclerosis of the vessel wall. It is frequently accompanied by calcification in the vessel wall. We report the case of a 61-year-old woman with idiopathic mesenteric phlebosclerosis. To our knowledge, this is the first case reported in Korea.
Calcinosis/diagnosis ; Colitis, Ischemic/diagnosis/etiology ; Colonoscopy ; Female ; Humans ; Mesenteric Vascular Occlusion/*diagnosis/etiology/pathology ; Mesenteric Veins/*pathology ; Middle Aged ; Sclerosis/pathology ; Tomography, X-Ray Computed

Idiopathic mesenteric phlebosclerosis, rare disease entity causing chronic mesenteric ischemia is a member of non-thrombotic, non-inflammatory stenosis or occlusion of the mesenteric veins. The histologic hallmark is marked fibrous mural thickening and sclerosis of the vessel wall. It is frequently accompanied by calcification in the vessel wall. We report the case of a 61-year-old woman with idiopathic mesenteric phlebosclerosis. To our knowledge, this is the first case reported in Korea.
Calcinosis/diagnosis ; Colitis, Ischemic/diagnosis/etiology ; Colonoscopy ; Female ; Humans ; Mesenteric Vascular Occlusion/*diagnosis/etiology/pathology ; Mesenteric Veins/*pathology ; Middle Aged ; Sclerosis/pathology ; Tomography, X-Ray Computed

Inflammatory pseudotumor of the lung is considered to be a rare, benign, neoplastic lesion, consisting mainly of spindle mesenchymal cells, sometimes in such a way that its histological appearance mimics that of a spindle cell sarcoma, fibrous histiocytoma or fibrosarcoma. A case of inflammatory pseudotumor of the lung occurring in a 13-year-old boy is reported with pathologic findings, including its ultrastructure. The patient had had no symptoms and accidentally discovered his condition after a chest X-ray examination at a regular school physical check up. The mass was located in the suprahilar area of the left lung. Exploratory thoracotomy revealed a large mass that was removed, together with the left upper lobe of the lung. Microscopically, the mass was composed of numerous interstitial inflammatory cells, mainly lymphoplasma cells. Ultrastructurally, the spindle-shaped mesenchymal cells were arranged haphazadly and the normal pulmonary structure was nearly totally destroyed. Emphasis is given to complete resection of the tumor for both diagnostic and therapeutic purposes.
Adolescent ; Child ; Fibrosarcoma ; Granuloma, Plasma Cell ; Histiocytoma, Benign Fibrous ; Humans ; Lung ; Male ; Plasma Cell Granuloma, Pulmonary* ; Sarcoma ; Thoracotomy ; Thorax

A congenital ureteral valve is a rare disease, with the first case presented in 1887, since when, only 42 cases have subsequently been reported. From a review of the reported cases, this abnormality was often found to be associated with other urological disorders, such as vesicoureteral reflux, ectopic ureter, complete and incomplete duplication of the kidney, and contralateral renal atrophy. Here, the case of an adult patient with multiple congenital ureteral valves and renal atrophy is reported.
Adult* ; Atrophy ; Constriction, Pathologic ; Humans ; Kidney ; Rare Diseases ; Ureter* ; Vesico-Ureteral Reflux

Primary cutaneous diffuse large B-cell lymphomas are characterized by a non-epidermotropic monotonous infiltrate of large follicular center cells with various proportions of centroblasts, large centrocytes, multilobated cells or immunoblasts without extracutaneous manifestations. We report a case of a 61-year-old man, who presented with several, annular plaques on the trunk. The clinicopathologic features and immunohistochemical profiles were diagnostic of primary cutaneous diffuse large B-cell lymphoma.
B-Lymphocytes* ; Humans ; Lymphoma, B-Cell* ; Middle Aged

BACKGROUND: Eosinophilic pustular folliculitis (EPF) is a rare chronic disease of unknown cause with pruritic papulopustular lesions and a prominent eosinophilic infiltrate. OBJECTIVE: The purpose of this study was aimed at evaluating the clinical and histopathological features of EPF. METHODS: The hospital charts and histopathologic slides of 8 patients with EPF diagnosed at Asan Medical Center from 1989 to 1998 were reviewed. We also reviewed the previously published reports of 10 patients with EPF in Korea. RESULTS: Nine male and nine female patients were enrolled in this study. The mean age at diagnosis of EPF was 26 years in men and 27 years in women. All the patients complained of mild to severe pruritus. EPF was presented with follicular papules or pustules, except 2 patients, who presented with erythematous plaques free of papules or pustules. All the patients showed the typical histopathological findings of EPF. Laboratory investigation showed hypereosinophilia in 73% of patients (11/15). The levels of blood eosinophils tended to decrease as cutaneous lesions resolved. EPF was improved with dapsone in 12 patients and the rest were treated with topical or systemic steroid or antihistamines. CONCLUSION: EPF may be more common in Korea than can be suspected. Because EPF showed characteristic histopathological findings and EPF responds well to dapsone, the knowledge with this disease may be important to diagnose and treat it.
Chronic Disease ; Chungcheongnam-do ; Dapsone ; Diagnosis ; Eosinophils* ; Female ; Folliculitis* ; Histamine Antagonists ; Humans ; Korea* ; Male ; Pruritus

More than 17 different terms, including carcinosatcoma and pseudosarcoma, have been applied to the rare polypoid tumors of the esophagus that demonstrate both carcinomatous and sarcomatous components. The multiplicity in terminology seems related to the uncertain histogenesis of these tumors. A demonstration of the ultrastructure of the spindle cells (containing desmosomes and tonofilaments) is consistent with an epithelial origin. The patient was a 53 year-old man who had suffered from dysphagia and foreign body sensation in larynx. Endoscopic finding was a large polypiod mass with ulceroinfiltrative lesion at the level of 27cm from the incisor. Pathologic findings were that the covering epithelium showed well differentiated squamous carcinoma with invasive pattern and the stroma contained islands of sarcoma and squamousl cell carcinoma. Immunoreactivity to cytokeratin was not observed. Partial esophagectomy and esophagogastrostomy was done. We report a case of rare malignant esophageal carcinosarcoma.
Carcinoma, Squamous Cell ; Carcinosarcoma* ; Deglutition Disorders ; Desmosomes ; Epithelium ; Esophagectomy ; Esophagus* ; Foreign Bodies ; Humans ; Incisor ; Intestines* ; Islands ; Keratins ; Larynx ; Middle Aged ; Sarcoma ; Sensation ; Stomach*