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.