Degos病一例并文献复习.docVIP

Degos病一例并文献复习 Degos病一例并文献复习 2010-05-25 武希润　李小峰　肖晋美　郭文栋　王来远 　　【摘　要】　目的　探讨Degos病病因、发病机制、临床表现、诊断及鉴别诊断。方法　报告具有特殊病症的皮肤病变伴小肠穿孔、溃疡病1例，分析其病变组织病理特 征，并复习有关文献。结果　除皮肤、皮下组织典型的楔形坏死外， 病变处血管内皮细胞增殖肿胀、管腔狭窄甚至闭塞，血管周围有多数淋巴细胞浸润。结论　Degos病是一种系统性坏死性血管炎。在临床上，对于皮肤病变合并 系统损害的病人，应考虑到Degos病。 　　【关键词】　Degos病(恶性萎缩性丘疹病)　　小肠穿孔　　血管炎 Degos′ disease:a case report and review of literature WU Xir un,LI Xiaofeng,XIAO Jinmei,et al.The Second Hospital,Shanxi Medical University,T aiyuan 030001 　　【Abstract】　Objective　To explore the etiology ,pathogenesis,clinical manifestation,diagnosis and differential diagnosis of Deg os′ disease.Methods　Pathognomonic skin lesions of Degos′disea se,accompanied by small bowel perforation and gastric ulcer in a 37-year-old woman,were reported. Histopathological features of skin lesions were studied.The literature related to Degos′ disease was reviewed.Results　The studies of skin histopothological study showed the typical wedge-shaped regions of necrobiosis and lymphocyte inflammatory infiltration.There were endothelial proliferation,swelling and fibrinoid necrosis in small dermal vessels with narrow ing of the lumina,thrombosis,obstruction,and perivascular lymphocyte infiltratio n in the subcutaneous arterioles of the skin lesions.Conclusion　Degos′disease is a systemic necrotizing vasculitis.Degos′disease should be c onsidered in the differential diagnosis of skin lesions associated with systemic involvement. 　　【Key words】　Degos′disease(malignant atrophic papulosis)　　s mall bowel perforation　　vasculitis 　　Degos病即恶性萎缩性丘疹病(malignant atrophic papulosis)是一种罕见、致死的坏死性 血管炎，主要表现为皮肤、胃肠道、中枢神经系统病变。本文报告一具有临床典型皮损、小肠穿孔、胃溃疡病例，并复习有关文献。 1　病历摘要 　　患者女性，37岁。主因躯干、四肢反复皮疹3年余，间断腹痛半年入院。患者于3年前躯干出 现淡红色丘疹，米粒样大小，散在分布，微痒继之丘疹扩大，中间凹陷呈瓷白色，周围隆起伴红晕，后变为皮肤瘢痕。之后皮疹分批出现，渐遍布于腹部、四肢、腰部、曾考虑结核性丘疹，予抗结核治疗，无明显效果。1998年4月出现间断脐周绞痛，疼痛不剧。1998年10月23日突感腹痛加剧，疼痛难忍，且迅速由中腹部、双下腹扩散至整个腹部，拍腹平片示膈下游离气体，遂剖腹探查。术中可见腹腔内有脓性渗出物，肠管表面有许多0.3 cm×0.5cm大小类似于皮肤改变的白色斑点，中间凹陷，距回盲部60 cm处小肠有一0.5 cm×0.5 cm大小穿孔，行穿孔修补术。术后仍感间断腹痛，右下腹为著。既往病人无关 节疼痛，无皮肤红斑、自