Chromobacterium violaceum (C. violaceum) is a gram-negative bacillus that is widespread in tropical and subtropical areas. Although C. violaceum rarely infects humans, it can cause critical illness with a mortality rate above 50%. Here, we report the successful treatment of a 15-year-old male who presented with bloodstream infection of C. violaceum along with sepsis, specific skin lesions, and liver abscesses. Cardiogenic shock induced by sepsis was reversed by venoarterial extracorporeal membrane oxygenation (VA ECMO). Moreover, C. violaceum-related purpura fulminans, which is reported herein for the first time, was ameliorated after treatment. This case report demonstrates the virulence of C. violaceum with the aim of raising clinical awareness of this disease.

Purpura fulminans is a rare and rapidly progressive septic process characterized by the development of hemorrhagic and ecchymotic lesions and skin necrosis. In this work, we report a case of a 52-year-old woman admitted to the Department of Emergency due to progressive purpura. The physical examination demonstrated a decreased skin temperature, unpalpable dorsalis pedis arteries, and ecchymoses covering both lower extremities. Laboratory tests indicated disseminated intravascular coagulation with prolonged activated partial thromboplastin time (APTT), low prothrombin time (PT), elevated d-dimer levels, and a low platelet count. A diagnosis of purpura fulminans was made, and steroids, therapeutic plasma exchange and empiric therapy, including antibiotic and anticoagulation therapy, were initiated immediately. Our treatment resulted in a good and sustained clinical response, as evidenced by the receding of blood blisters and the normalization of the patient\'s coagulation factors, but bilateral below-knee amputation was inevitable. Finally, the patient recovered well and was discharged home without any complications other than amputation.

UNASSIGNED: Severe complications may cause a fatal or disabling outcome in patients with Rickettsia japonica infection but are poorly understood.
UNASSIGNED: We identified 11 patients with only Rickettsia japonica infection with metagenomics next generation sequencing (mNGS) during April to November 2021 at Yichang Central People\'s Hospital, China. Clinical data were obtained through review of medical records.
UNASSIGNED: Most patients realized that they had symptoms about one or two days after being bitten. Fever (91%), pulmonary effusion (91%), rash or erythema (100%), abnormal urine (100%), neutropenia (100%), lymphopenia (100%), and thrombocytopenia (100%) were the most common clinical signs. Six severely ill patients were admitted to the intensive care unit and five had mild symptoms. Systemic manifestations such as vomiting (83%), neurological manifestations (100%), and disseminated intravascular coagulation (100%) were more frequently observed in the severe cases, 33.3% of whom developed purpura fulminans requiring amputation or skin graft, and 16.6% died two days after admission. Some patients experienced sequelae.
UNASSIGNED: Our study found that patients with critical Rickettsia japonica infection complicating disseminated intravascular coagulation had high risk of poor outcome.

BACKGROUND: Purpura fulminans (PF) is a hematological emergency that can be caused by severe congenital protein C (PC) deficiency. It has been rarely reported in the Chinese population. We aimed to characterize the clinical and genetic features of Chinese pediatric patients with severe congenital PC deficiency who first presented with PF.
METHODS: Twelve pediatric patients were diagnosed with severe congenital PC deficiency with PF, which was diagnosed based on our hospital records and previous reports from 1988 to July 2021 in China. We evaluated the clinical and genetic features of these patients.
RESULTS: Nine patients (9/12, 75%) had onsets that were observed within the first 48 h after birth. Six patients had a family history of thromboembolism. There was no consanguinity. Other symptoms were intracranial thrombosis or hemorrhage (4, 33.3%), ocular lesions (2, 16.7%), gastrointestinal hemorrhage (2, 16.7%) and kidney infarction before birth (1, 8.3%). All but one of the patients (one case not detected) had a plasma PC activity of <10%. The genetic study indicated that in the eight patients with inherited PC deficiency, two were homozygous, five were compound heterozygous and one was heterozygous for PC deficiency.
CONCLUSIONS: This is the first and largest case series of Chinese pediatric patients with severe congenital PC deficiency who first presented with PF. It has been shown that treatment with both fresh frozen plasma and anticoagulants is recommended when PC concentrate is not easily available, especially in developing countries.

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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.

UNASSIGNED: Our objective was to study the phenotype of and molecular genetic mechanisms underlying congenital protein C (PC) deficiency in Chinese neonates. We report the case of a neonate who presented 4 h after birth with purpura fulminans of the skin and thrombosis in the kidney. We also carried out a through literature review to study the genotype and phenotype, relevance, diagnosis, management, and prognosis of neonates with congenital PC deficiency in China.
UNASSIGNED: Following a septic work-up and check of PC and protein S (PS) levels that showed PC deficiency, we investigated the patient\'s and her parents\' genotypes. Our patient was found to have a plasma PC level of 0.8%. Molecular testing revealed a compound heterozygous mutation of the PROC gene: From the father, a c._262 G > T p. ASP88Tyr mutation in exon 4; from the mother, a C. 400 + 5G mutation in intron 5 that had been previously reported as likely pathogenic. Both parents were found to have heterozygous mutations for PC deficiency. In China, 5 other cases of congenital PC deficiency in the neonatal period were reported in the literature. In those cases, purpura fulminans and thrombosis were the main symptoms, and homozygous or compound heterozygous mutations of the PROC gene were identified.
UNASSIGNED: Congenital PC deficiency should be ruled out for neonates presenting with purpura fulminans and thrombosis.

Purpura fulminans (PF) is a neonatal presentation of homozygous or compound heterozygous protein C (PC) deficiency; infants who are diagnosed with it are determined to have a major defect in coagulation regulation which is associated with undetectable levels of PC. We report a pedigree who suffered from the hereditary PC deficiency with compound heterozygous mutants; genetic analysis revealed compound heterozygous mutations of 262 G > T(Asp88Tyr) and 400 + 5G > A that were identified in the proband; moreover, Asp88Tyr and 400 + 5G > A were also detected in the father and the mother, respectively. A bioinformatics analysis revealed 262 G > T is probably damaging, and structural analysis indicated a possible mechanism for the functional impairment of PC in this pedigree.

暂无摘要。

Objective: To improve the understanding of the rare clinical presentation and management of purpura fulminans (PF) in patients with paroxysmal nocturnal haemoglobinuria (PNH). Methods: A case of PF occurring in PNH is reported, while the related literature review is conducted. Results: A 49-year-old male patient suffered from one-week history of fever, greenish-brown colour urine, multiple well demarcated and painful purpura of the head and neck. He had been reported to have two thromboembolic events during the 22-year course of PNH. Skin biopsy displayed classic PF features. Laboratory testing showed a high PNH clone, intravascular hemolysis and coagulation system changes. After sufficient anticoagulation and short course of glucocorticoid therapy, the clinical conditions were improved correspondingly. During a follow-up period of 6 month, there was no recurrence of thrombosis. Conclusion: PF should be considered in PNH patients with unexplained, quickly developed painful purpura. Extensive work-up should be performed to find out other potential thrombophilic risk factors after diagnosis of PF. Early diagnosis, adequate anticoagulation therapy and control hemolysis were essential to PF treatment occurring in PNH. The survival of patients and the qualities of life can be improved. The PNH clone detection is needed to evaluate the status of procoagulation and predict the risk of recurrent thrombosis.
目的： 提高对于阵发性睡眠性血红蛋白尿症（PNH）合并暴发性紫癜这一罕见部位血栓并发症的认识并探讨对此类急重症的诊治。 方法： 报道一例PNH患者合并暴发性紫癜的临床诊治过程，并对相关文献进行复习。 结果： 患者，男，49岁，诊断PNH 22年，曾发生下肢静脉及门静脉血栓，因门静脉血栓迁延继发门脉高压、胃底静脉曲张、血小板减少，本次主要表现为前驱感染后多处皮肤大片痛性紫黑色皮疹、尿色加深，经皮肤活检病理诊断为暴发性紫癜，检测提示PNH克隆高负荷、血管内溶血加重，经依诺肝素6 000 U每12 h 1次抗凝、头孢美唑抗感染及短程泼尼松控制溶血等治疗后，皮肤梗死得以控制好转，随访6个月病情稳定，无再次血栓事件发生。 结论： PNH患者如发生原因不明的急性痛性紫黑色皮疹，需高度警惕暴发性紫癜，同时应积极寻找其他易栓因素并评估体内凝血状态。早期诊断并给予充分的抗凝、控制溶血是PNH伴发暴发性紫癜的治疗关键，也有助于改善患者的预后。在临床诊疗中也应重视PNH克隆负荷检测，有助于评估患者易栓状态、预测血栓发生风险并调整抗凝策略。.