Purpose

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.

Methods

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).

Results

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.

Conclusion

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.

中文翻译：

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老年人库欣综合征罕见并发症一例报告

目的

库欣综合征 (CS) 是一种罕见且严重的疾病，由持续性皮质醇增多症引起。CS的临床表现在老年人中可能不典型，这些患者的诊断常常被漏诊。传染性暴发性紫癜 (PF) 是一种危及生命的血栓形成的弥散性血管内凝血，死亡率高。据我们所知，以前没有报道过 CS 患者发生 PF。

方法

我们描述了一名 84 岁女性，感染严重，但体温正常。她患有多种疾病，尤其是性格改变。体格检查发现皮肤薄，全身水肿，多发散在瘀斑。结合血清皮质醇明显升高（36.85 ug/dl）和肾上腺CT扫描显示腺瘤，诊断为内源性CS。住院期间，患者右侧大腿和背部出现严重皮下出血。皮肤活检显示多处小血管血栓形成，表明患者出现了罕见的 CS 并发症，暴发性紫癜 (PF)。

结果

慢性皮质醇增多症可导致免疫抑制、低度炎症、内皮损伤和高凝状态，这些共同增加了 PF 的易感性。CS合并感染性肺纤维化时，液体复苏、抗生素、输注血制品、清创是有效​​的治疗措施。

结论

CS患者尤其是老年人可发生PF导致的严重皮下出血。临床医生应警惕认知能力下降和性格改变的老年人 CS 的诊断。