purpura fulminans
Introduction
Introduction to fulminant purpura PurpuraFulminans is a fatal hemorrhagic emergency that often has a history of infection before onset. The disease is rapidly onset and is characterized by hemorrhagic necrosis, hypotension, fever, and disseminated intravascular coagulation (DIC). The disease was first described by Guelliot in 1884. Newborn fulminant purpura is likely to be fatal. The homologous protein C or S deficiency, meningococcal meningitis, etc. can cause this disease. If not treated, it can quickly kill. basic knowledge The proportion of illness: 0.0003% Susceptible people: good for children Mode of infection: non-infectious Complications: disseminated intravascular coagulation
Cause
The cause of fulminant purpura
Bacterial and viral infections (30%):
The cause is unknown, may be related to vascular endothelial cell damage caused by bacterial and viral infections, most commonly in the recovery period of childhood infections, such as scarlet fever, streptococcal pharyngitis and tonsillitis, meningococcal meningitis, chickenpox, measles, contagious Hepatitis, subacute bacterial endocarditis, sepsis, miliary tuberculosis and typhus, but there are no pre-existing conditions.
The antigen of the bacterial endotoxin itself sensitizes the vascular endothelium (20%):
Due to high fever, microinfarct thrombosis, toxins, septic embolism, vascular endothelial cell damage and disseminated intravascular coagulation (DIC), which in turn leads to depletion of platelets, fibrinogen, prothrombin and other clotting factors Diffuse ecchymosis occurs, and patients who develop after the infectious disease may also be caused by the antigen of the bacterial endotoxin itself sensitized the vascular endothelium. This disease has extensive thrombosis and perivascular inflammatory infiltration and is considered to be a kind of disease. Shwartzman phenomenon.
Pathological changes (15%):
Pathologically, the epidermis and part of the dermis are often extensively necrotic. The epidermis of the bullous area is separated from the dermis. The vascular embolism in the dermis near the necrotic area. The embolus consists of platelets, fibrin, etc. There is no inflammatory reaction in the blood vessels, and there is a large amount of bleeding in the dermis.
Prevention
Fulminant purpura prevention
1. To prevent possible incentives, the room should not be too cold and humid, and the temperature should be appropriate.
2. Strengthen nutrition, not cold drink and over-eating fat and sweet taste, avoid spicy food and avoid tobacco, wine.
3. Early diagnosis, early treatment, active treatment of the primary disease.
Complication
Fulminant purpura complications Complications disseminated intravascular coagulation
Sometimes it can cause ischemic gangrene in the extremities. Post-natal accidents can be complicated by defibrination syndrome.
1. After tissue necrosis, due to the infection of secondary spoilage bacteria and other factors, it presents a special morphological change such as black and dark green, called gangrene.
2. Disseminated intravascular coagulation (DIC) is a disease that occurs on the basis of many serious diseases or under certain special conditions to activate the human coagulation system, leading to microcirculation diffuse microthrombus formation and subsequent Syndrome of fibrinolysis. Due to the different degrees of DIC factor consumption, the consumption of DIC decreased.
Symptom
Symptoms of fulminant purpura Common symptoms chills, freckle shock, limb ischemia, coma, high fever
Pediatric-based, occurring in 2 to 4 weeks after various bacteria and viral infections, the incidence is rapid, the condition is sinister, most of them are fatal, often occur in the lower limbs, but the hips, torso and facial couples can also be involved, showing a symmetric distribution, which is a large touch. Painful ecchymosis, tends to fuse, hemorrhagic bullae and coagulative necrosis on the ecchymosis, the edge is separated from normal skin, the base and the subcutaneous fibrous tissue are adhered, the surface is covered with thick black sputum, the latter is not easy to peel, sometimes Causes ischemic gangrene of the extremities and requires amputation, often accompanied by severe systemic poisoning symptoms such as high fever, chills, collapse, etc., resulting in rapid entry into shock state within 2 to 3 days, coma, failure or cerebral hemorrhage and eventually death, kidney, intestinal lung, etc. It can also be affected at the same time, and there is an autopsy to find adrenal hemorrhagic necrosis as a symptom of Waterhouse-Friderichsen syndrome.
Examine
Examination of fulminant purpura
Normal or decreased platelets, anemia, leukocytosis, prolonged clotting time, consumption of various coagulation factors such as thrombin, prothrombin, and thrombin, positive for protamine coagulation test.
Diagnosis
Diagnosis and identification of fulminant purpura
Most of the patients are children, which are fulminant, generalized, tender and extensive ecchymosis, rapid progress, and sinister conditions. If it occurs after infection, it is more helpful for diagnosis.
This disease is the result of disseminated intravascular coagulation. If it is possible to repeatedly (continuously) measure factor I (fibrinogen), II (prothrombin), V, VIII and VII-X complexes, estimate the condition, evaluate Efficacy is extremely important, and occasional hematology results are normal.
Defibrination syndrome, which is associated with postpartum accidents, should be differentiated from allergic purpura. The latter should be differentiated from allergic purpura. The latter is scattered with hemorrhagic papules or ecchymoses, often accompanied by joint and abdominal symptoms. Therefore, it can be identified.
