A Case of Gastric Adenocarcinoma in a Patient with X-linked Agammaglobulinemia / 대한소화기내시경학회지

X-linked agammaglobulinemia is a common type of primary immunodeficiency disorder that's caused by mutation of the BTK gene. The absence of B lymphocytes and plasma cells causes recurrent infections. Patients with X-linked agammaglobulinemia also have a high risk for developing hematological malignancies and, to a lesser degree, carcinoma. We report here on a 26-years-old male patient who suffered with X-linked agammaglobulinemia that was caused by BTK gene mutation, and he developed a gastric cancer in the antrum. He was noted to have chronic atrophic gastritis and diffuse intestinal metaplasia on the endoscopic examination that was done 7 years previously. We recommend regular esophagogastroduodenoscopic evaluation for a patient with X-linked agammaglobulinemia in order to make an early diagnosis of stomach carcinoma.

A Case of Degos' Disease Presenting with Abdominal Angina and Widespread Skin Lesions

Malignant atrophic papulosis (MAP), also known as Degos' disease, is a rare and often fatal occlusive thrombotic vasculopathy, with fewer than seven cases reported in Korea. MAP is characterized by porcelain-white, atrophic, papular skin lesions and multi-organ system involvement, especially the gastrointestinal (GI) tract and nervous system. Involvement of the GI tract is usually associated with a poor prognosis. To date, no treatment has been shown to be effective in the treatment of MAP. We describe a 52-year-old man who presented with a 5-month history of abdominal angina and a 2-year history of multiple skin lesions on the trunk and extremities. The skin lesions were papules, 4-6 mm in diameter, with a porcelain-white center and a slightly raised erythematous telangiectatic rim. A biopsy of a skin lesion showed a wedge-shaped degeneration of collagen in the dermis and atrophic epidermis. An explorative laparoscopy revealed multiple, yellow-white plaques scattered throughout the small bowel. A biopsy of the small bowel showed sclerotic vascular alterations containing intravascular fibrin thrombi. He was started on aspirin (100 mg daily) and has survived for 24 months since the onset of gastrointestinal symptoms.

A Case of Degos' Disease Presenting with Abdominal Angina and Widespread Skin Lesions

Malignant atrophic papulosis (MAP), also known as Degos' disease, is a rare and often fatal occlusive thrombotic vasculopathy, with fewer than seven cases reported in Korea. MAP is characterized by porcelain-white, atrophic, papular skin lesions and multi-organ system involvement, especially the gastrointestinal (GI) tract and nervous system. Involvement of the GI tract is usually associated with a poor prognosis. To date, no treatment has been shown to be effective in the treatment of MAP. We describe a 52-year-old man who presented with a 5-month history of abdominal angina and a 2-year history of multiple skin lesions on the trunk and extremities. The skin lesions were papules, 4-6 mm in diameter, with a porcelain-white center and a slightly raised erythematous telangiectatic rim. A biopsy of a skin lesion showed a wedge-shaped degeneration of collagen in the dermis and atrophic epidermis. An explorative laparoscopy revealed multiple, yellow-white plaques scattered throughout the small bowel. A biopsy of the small bowel showed sclerotic vascular alterations containing intravascular fibrin thrombi. He was started on aspirin (100 mg daily) and has survived for 24 months since the onset of gastrointestinal symptoms.

A Case of Endometriosis with Chylous Ascites / 대한소화기내시경학회지

Chylous ascites is the extravasation into the peritoneal cavity of milky chyle that is rich in triglycerides. Sixty to seventy percent of chylous ascites cases are caused by cirrhotic liver disease, congestive heart failure, or malignancy. The most common malignancy associated with chylous ascites is lymphoma. Rarely, tuberculosis has been reported as a cause of chylous ascites. Appropriately, lymphoma, ovarian cancer or tuberculosis is considered first in the differential diagnosis when a young woman of reproductive age presents with chylous ascites. Recently, we examined a 26-year-old woman who visited our hospital because of abdominal distension. Ascites tapping, esophagogastroduodenoscopy, colonoscopy and diagnostic laparoscopic biopsy led to a diagnosis of intraperitoneal endometriosis with chylous ascites. To date, some cases of endometriosis complicated by massive ascites have been reported. However, endometriosis complicated by chylous ascites has not been previously reported. The case was successfully treated using a gonadotropin-releasing hormone agonist and diuretics.

A case of mammary-like glands carcinoma of the vulva / 대한내과학회지

Mammary-like glands are poorly recognized structures of normal vulvar skin. Here, we report the first case of primary mammary-like gland adenocarcinoma of the vulva in Korea and discuss the differential diagnosis and treatment of this rare tumor. A 62-year-old woman presented with an itching sensation and palpable mass of the vulva. Abdominal computed tomography showed multiple variable-sized nodules with peripheral rim enhancement in the liver. The pathology of the vulva and liver lesions was consistent with invasive ductal carcinoma of the breast, with positive staining for estrogen receptors, progesterone receptors, HER-2, and GCDFP-15. Ultrasonography and magnetic resonance imaging of the breast were normal. Given the histological findings in conjunction with the absence of a breast lesion, a diagnosis of primary mammary-like gland adenocarcinoma of the vulva was made. She died of pneumonia 7 weeks after the diagnosis.

A case of pylephlebitis with Streptococcus viridans and Bacteroides fragilis bacteremia secondary to diverticulitis / 대한내과학회지

Pylephlebitis, a septic thrombophlebitis of the portal vein or one of its tributaries, is a life-threatening complication of intra-abdominal infection. The causes of pylephlebitis include acute diverticulitis, appendicitis, acute cholecystitis, necrotizing pancreatitis, inflammatory bowel disease, and bowel perforation. Although pylephlebitis is an unusual complication of diverticulitis, its morbidity and mortality remain high. Therefore, early diagnosis and initiation of adequate antibiotic therapy is important for improving the long-term prognosis of patients suffering from this rare disease. We report a case of pylephlebitis with Streptococcus viridans and Bacteroides fragilis bacteremia secondary to diverticulitis with a review of the literature.

A Case of Posttraumatic Pleural Effusion with Peripheral Eosinophilia / 결핵및호흡기질환

Eosinophilic pleural effusions (EPE) are defined as those effusions that contain at least 10% eosinophils, and EPE can be associated with peripheral blood eosinophilia in a variety of systemic diseases. There have been a few cases that have addressed the association of peripheral blood eosinophilia and posttraumatic EPE, and this condition can be misdiagnosed as being the result of other causes due to the delayed presentation. We report here on a case of 47-year-old male who presented with eosinophilic pleural effusion associated with peripheral blood eosinophilia at 2 months after minor chest trauma. We excluded the other possible causes such as consumption of drugs, parasite infection, malignancy, diseases of pulmonary eosinophilic infiltration, autoimmune diseases and pulmonary thromboembolism. We observed his clinical course without specific treatment. Three months later, the pleural effusion completely disappeared and the number of peripheral eosinophils returned to normal.

Volatile Sulfur Compounds as a Predictor for Esophagogastroduodenal Mucosal Injury

BACKGROUND/AIMS: Halitosis is a symptom that bothers patients more socially than medically and its pathogenic mechanisms are unclear and treatment armamenterium is limited. Clinicians generally ignored active interventions. Since halitosis is closely associated with volatile sulfur compounds (VSCs), we used a Halimeter and gas chromatography to measure VSCs in patients with Helicobacter-pylori (H. pylori)-associated gastric diseases. METHODS: We categorized 72 patients with H. pylori infection into two groups based on their endoscopic findings: a non-erosive mucosal group (NE, n=24) and an erosive mucosal group (E, n=48). Halitosis was objectively assessed by applying either a Halimeter to breath air or gas chromatography to gastric juice. Simultaneously, the expression of VSC-generating enzyme was measured with reverse-transcriptase PCR using mRNA isolated from biopsy tissues. RESULTS: The levels of VSCs in exhaled breaths or aspirated gastric juices differed significantly between the NE and E groups (p<0.00001), suggesting that VSCs might reflect eroded epithelial damage induced by H. pylori infection. The expressions of cystathionine beta-synthase (CBS) and cystathionine gamma-lyase (CSE) were broadly consistent with the degree of mucosal injury. CONCLUSIONS: Erosive changes in esophagogastroduodenal mucosa were strongly correlated with increased VSC levels, suggesting that halitosis might result from H. pylori-associated erosive lesions.