ABSTRACT
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.
Subject(s)
Humans , Middle Aged , Aspirin , Biopsy , Collagen , Dermis , Epidermis , Extremities , Fibrin , Gastrointestinal Tract , Korea , Laparoscopy , Malignant Atrophic Papulosis , Nervous System , Prognosis , SkinABSTRACT
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.
Subject(s)
Humans , Middle Aged , Aspirin , Biopsy , Collagen , Dermis , Epidermis , Extremities , Fibrin , Gastrointestinal Tract , Korea , Laparoscopy , Malignant Atrophic Papulosis , Nervous System , Prognosis , SkinABSTRACT
Primary cardiac tumors are rare findings and they are mostly benign. Malignant primary cardiac tumors make up 25% of the primary cardiac tumors and most of them are sarcomas. Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma in late adulthood, and it is an extremely rare sub-type of cardiac sarcoma. It is known that MFH displays a poor clinical course with complete resection being the most important prognostic factor for survival. We described here the case of a malignant fibrous histiocytoma of the heart in a 54-year-old woman who presented with dyspnea. The tumor was treated by mass excision via cardiac explantation and autotransplantation followed by adjuvant chemotherapy. This is the first report of cardiac autotransplantation in Korea.