Stejskalová A, Stanová M, Vosmík F
Hlavův I. patologicko-anatomický ústav fakulty vseobecného lékarství Univerzity Karlovy, Praha.
Sb Lek. 1990 Jan;92(1):1-5.
In a 49-year-old female patient with an eruption of lentil-shaped papules on the trunk and extremities, persisting for six years, originally in another department scleroderma guttata was diagnosed. For one year the patient suffered also from dyspeptic complaints, loss of weight and relapsing polyserositis. The complaints receded temporarily after immunosuppressive therapy. On surgical revision of the abdominal cavity on account of serous peritonitis during the last three months eruption of whitish foci on the intestinal serosa and omentum was found. Bioptic excision of the skin revealed an old bland infarct. From the clinical course and dermatological picture papulosis maligna atrophicans (Degos' syndrome) was diagnosed. Autoptic examination confirmed the clinical diagnosis of the syndrome by the finding of thromangiitis of Bürger's type with bland infarcts of the small intestine and perforation of the jejunum.
一名49岁女性患者，躯干和四肢出现扁豆状丘疹，持续六年，最初在另一科室被诊断为点滴状硬皮病。患者一年来还伴有消化不良症状、体重减轻和复发性多浆膜炎。免疫抑制治疗后症状暂时缓解。因过去三个月出现浆液性腹膜炎而进行腹腔手术探查时，发现肠浆膜和网膜上有白色病灶。皮肤活检切除显示陈旧性非特异性梗死。根据临床病程和皮肤表现，诊断为萎缩性恶性丘疹病（德戈斯综合征）。尸检发现有布格尔型血栓性血管炎，小肠有非特异性梗死及空肠穿孔，证实了该综合征的临床诊断。
