Dermatological Lesions of Cholesterol Embolisation Syndrome and Kaposi Sarcoma Mimic Primary Systemic Vasculitis: Case report study.

Primary systemic vasculitis can present with a wide spectrum of manifestations ranging from systemic non-specific features such as fever, malaise, arthralgia and myalgia to specific organ damage. We describe two cases of cholesterol embolisation syndrome and Kaposi sarcoma mimicking primary systemic vasculitis, both of which were characterised by features such as livedo reticularis, blue toe syndrome, a brown purpuric skin rash and positive perinuclear anti-neutrophil cytoplasmic antibodies associated with Kaposi sarcoma. Establishing the right diagnosis was challenging and thus this report aimed to highlight the possible ways to distinguish them from primary systemic vasculitis.

Síndrome del dedo azul / Blue finger syndrome

El síndrome o signo del dedo azul (SDA) es una entidad poco frecuente causada por la oclusión de vasos periféricos distales. Se manifiesta como una coloración inicialmente azulada de uno o más dedos, referidos primero en las extremidades inferiores, en ausencia de traumatismo previo, y de etiología múltiple. La importancia de establecer un diagnóstico temprano y tratamiento oportuno es evitar la evolución extrema de necrosis o la pérdida de la vida del paciente.

The blue finger syndrome (or sign) is a rare entity caused by distal occlusion of peripheral vessels, which initially manifested as bluish discoloration of one or more fingers, first descriptions described in lower extremities in the absence of previous trauma of multiple etiologies; the importance of early diagnosis and treatment to prevent extreme changes in necrosis or loss of patient life.

[Progressive renal failure in spontaneous-onset cholesterol crystal embolism]. / Progrediente Niereninsuffizienz bei spontan auftretenden Cholesterinkristallembolien.

CASE REPORT: A 69-year-old man was admitted to the authors' hospital with an increase of plasma creatinine from 1.4 up to 4.9 mg/dl within 4 months and the clinical complaints of painful purple toes, recurrent epistaxis and disturbances of equilibrium. His past medical history was remarkable for three transient ischemic attacks and the diagnosis of a metabolic syndrome. Magnetic resonance imaging showed vasculitis-like lesions in the brain. Eosinophilia and tubular proteinuria were detected. Renal insufficiency was caused by cholesterol crystal embolism, as shown both by skin and renal biopsy. Aortic plaques were identified as the putative source of cholesterol embolization. CONCLUSION: In case of rapidly progressive renal failure, cholesterol crystal embolism must be considered even without preceding angiography.

Native superficial femoral artery peripheral atherectomy site pseudoaneurysm: a case report.

A 45-year-old woman who presented with blue toe syndrome was treated with atherectomy for a focal plaque located in the superficial femoral artery. She subsequently developed a large pseudoaneurysm at the atherectomy site requiring multiple sequential endovascular procedures in order to maintain in-line blood flow to the foot. Pseudoaneurysm formation at native peripheral artery atherectomy site has not been reported previously. We discuss possible complications of atherectomy and the possible mechanism of pseudoaneurysm formation after atherectomy. We address the importance of understanding risks of these minimally invasive procedures along with planning follow-up duplex and potential bail-out tactics.

A 77-year-old woman with sudden onset of blue discolouration of right third toe.

We report on a 77-year-old woman with a history of peripheral vascular disease who presented with an acute-onset tender blue toe and deteriorating renal function. A clinical diagnosis of blue toe syndrome was made but the patient deteriorated rapidly and died. This case illustrates the rapidly devastating nature and fatality of blue toe syndrome. There is no effective treatment for this condition.

Doppler ultrasound monitoring for detection of microembolic signals in peripheral arteries.

OBJECTIVE: to use Doppler ultrasound to detect peripheral microemboluation. METHODS: standard Transcranial Doppler equipment was used to peripheral detect peripheral embolic high intensity transient signals (HITSs) in a pig model following injection of microparticles and atheroma, and in 23 patients who underwent open repair of an abdominal aortic aneurysm (AAA), six patients with blue toe syndrome and 10 age matched healthy subjects. RESULTS: the pig study showed increasing signal intensity with particle size. Particles of 100 (n=24), 200 (n=17), and 400 microm (n=31) elicited 14, 25, 33 dB signals, respectively (p<0.05). During AAA surgery, the intensity (median) of HITSs before clamping (n=226) and after declamping (n=1216) were 14, and 20dB, respectively (p<0.001). Quite a few HITSs were detected after surgery. In patients with blue toe syndrome, a total of 63 HITSs could be detected, and the frequency of HITSs (median: 5.72/30min) was significantly higher than that in patients with AAA before surgery (0.065/30min) (p<0.001). CONCLUSIONS: Doppler ultrasound technique may be a clinically useful test to guide the treatment of patients at risk of distal atheroembolic events.

Embolismo por cristales de colesterol, con fracaso renal agudo o subagudo, diagnosticado por las lesiones cutáneas / Cholesterol crystal embolism, with acute or subacute renal failure, diagnosed by cutaneous manifestations

El embolismo por cristales de colesterol (ECC) es una complicación de la enfermedad arterioesclerótica en la que el desprendimiento de fragmentos de placas de ateroma, principalmente de grandes arterias, provoca oclusión de pequeños vasos. Puede desencadenarse espontáneamente, tras maniobras vasculares invasivas o tras terapéuticas anticoagulantes/fibrinolíticas. Presentamos cinco pacientes con ECC, manifestado en forma de insuficiencia renal aguda o subaguda, diagnosticado a través de las lesiones cutáneas acompañantes. Todos tenían factores de riesgo para ateromatosis; en cuatro hubo factores desencadenantes y en un caso el embolismo fue espontáneo. Todos los pacientes se encuentran vivos hasta la fecha aunque con secuelas de intensidad variable. El ECC es un proceso multisistémico, grave, en el que puede afectarse cualquier órgano. Debe sospecharse en todo paciente de edad avanzada con factores cardiovasculares de riesgo que haya sido sometido a un agente precipitante y/o desarrolle fallo renal acompañado de lesiones cutáneas características. Debido a la frecuencia de las manifestaciones cutáneas y a la importancia de un diagnóstico y tratamiento precoz es fundamental la presencia del dermatólogo en el diagnóstico de estos procesos (AU)

Multiple spontaneous small bowel perforations due to systemic cholesterol atheromatous embolism.

A-65-year-old man was admitted for coronary and peripheral angiography to evaluate angina pectoris and peripheral vascular disease. Following angiography, he suffered from blue toes, livedo reticularis and progressive renal failure. The patient's condition continued to deteriorate, including the development of malnutrition. Four months later he suddenly developed panperitonitis, went into shock and died. The autopsy verified multiple perforations of the small bowel with disseminated cholesterol atheromatous embolism. The other organs including kidney were also invaded by atheroembolism. This was a rare case of multiple spontaneous perforations of small bowel due to systemic cholesterol atheromatous embolism.

Blue toe syndrome associated with rapidly progressive glomerulonephritis: ultimately revealed essential mixed cryoglobulinemia.

The blue toe syndrome is a rare presentation in a number of medical disorders. We report a 35-year-old woman who initially presented with blue toe syndrome and rapidly progressive glomerulonephritis. Essential mixed cryoglobulinemia with vasculitis and renal failure was documented by laboratory tests and renal biopsy. She was on maintenance hemodialysis as renal failure persisted after steroid and immunosuppressive agents therapy. Her gangrenous changes of bilateral toes were autoamputated symmetrically and uneventfully.