Notash Ali Y, Mazoochy Hamed, Mirshams Mostafa, Nikoo Azita
Department of Surgery, Sina Hospital, School of Medical Sciences, University of Tehran, Tehran, Iran.
Saudi Med J. 2008 Jan;29(1):133-7.
Degos disease DD is a rare obstructive vasculopathy characterized by distinctive skin lesions. Involvement of the soles, palms and genitalia is rare. In most cases, disease has an unfavorable course and involves gastrointestinal tract, central nervous system and occasionally other organs. Pleural and pericardial involvements are usually minor manifestations with prolonged course. Death occurs in approximately 50% of the patients usually due to intestinal perforation or central nervous system bleeding. We describe a 48-year-old man of lethal systemic DD. Widespread skin lesions with involvement of palmoplantar surfaces, genitalia and scalp were ignored for 3 years, whereas the disease revealed own malignant nature. The disorder progressed to nervous, gastrointestinal and cardiopulmonary system that led to death after 5 months from onset of systemic involvement as severe restrictive cardio-pulmonary insufficiency. Autopsy showed diffuse fibrotic changes in serosal membranes and internal organs.
迪戈斯病（DD）是一种罕见的阻塞性血管病，其特征为独特的皮肤病变。累及足底、手掌和生殖器的情况罕见。在大多数病例中，该病病程不佳，累及胃肠道、中枢神经系统，偶尔还累及其他器官。胸膜和心包受累通常是病程较长的轻微表现。约50%的患者会死亡，通常是由于肠穿孔或中枢神经系统出血。我们描述了一名患有致死性系统性迪戈斯病的48岁男性。广泛的皮肤病变累及掌跖面、生殖器和头皮，被忽视了3年，而该病显示出其恶性本质。该疾病进展至神经、胃肠道和心肺系统，在全身受累开始5个月后因严重的限制性心肺功能不全导致死亡。尸检显示浆膜和内脏有弥漫性纤维化改变。
