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

Bon-Ho Ku, Yeun Jung Lim, Hyo Kyung Choi, Young Ho Kim, Jongwook Park, Chi Hoon Kim, Tae-Hoon Jang, Yong Woo Seo, Young Cheol Jo, Kee-Taek Jang, Chang Ohk Sung, Dong-Kyung Chang
2010 Intestinal Research  
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
more » ... escribe 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. (Intest Res 2010;8:70-74)
doi:10.5217/ir.2010.8.1.70 fatcat:plnkb4nspnhnrav35n2wjaidy4