库欣综合征(CS)是由持续皮质醇增多症引起的一种罕见且严重的疾病。老年人CS的临床表现可以不典型,这些患者的诊断经常被遗漏。传染性紫癜(PF)是一种威胁生命的疾病,弥散性血管内凝血的血栓形成,死亡率高。据我们所知,以前没有报道过CS患者发生PF。
我们描述了一名84岁女性出现严重感染,但温度正常.她患有多种疾病,尤其是性格改变。体格检查显示皮肤薄,一般水肿,和多发性散见性瘀斑。合并血清皮质醇明显升高(36.85ug/dl),肾上腺CT扫描显示腺瘤,内源性CS被诊断。住院期间,患者右大腿和背部出现严重皮下出血。皮肤活检显示多个小血管血栓形成,提示患者出现罕见的CS并发症,PurpuraFulminans(PF)。
慢性皮质醇增多症可引起免疫抑制,低度炎症,内皮损伤,和高凝状态,它们一起增加了PF的易感性。液体复苏,抗生素,输注血液产品,CS合并感染性PF时,清创是有效的治疗措施。
CS患者可发生PF引起的严重皮下出血,尤其是老年人。临床医生应警惕认知衰退和人格改变的老年人CS的诊断。
Cushing\'s syndrome (CS) is a rare and severe disease caused by sustained hypercortisolism. The clinical manifestations of CS can be atypical in the elderly, and the diagnosis in these patients is often missed. Infectious Purpura Fulminans (PF) is a life-threating, thrombotic form of disseminated intravascular coagulation with high mortality. To our knowledge, PF occurring in a patient with CS has not been reported previously.
We described an 84-year-old female presented with severe infection, but normal temperature. She suffered from a variety of diseases especially personality change. Physical examination revealed thin skin, general edema, and multiple scattered ecchymosis. Combined with obviously elevated serum cortisol (36.85 ug/dl) and adenoma revealed by adrenal CT scanning, endogenous CS was diagnosed. During hospitalization, the patient developed serious subcutaneous hemorrhage on the right thigh and back. The skin biopsy showed multiple small vessel thrombosis suggesting that the patient developed the rare complication of CS, Purpura Fulminans (PF).
Chronic hypercortisolism can cause immune suppression, low-grade inflammation, endothelial damage, and a hypercoagulable state, which together increased susceptibility of PF. Fluid resuscitation, antibiotics, infusion of blood product, and debridement were effective treatment measures when CS complicated with infectious PF.
Severe subcutaneous hemorrhage due to PF could occur in the patients of CS, especially in the elderly. Clinicians should be alert to the diagnosis of CS in older adults with cognitive decline and personality change.