No abstract available.
Protein C Deficiency* ; Protein C* ; Purpura Fulminans* ; Purpura*

We experienced with a death by the reason of sudden development of purpura fulminans during the illness of measles in 28 months old boy, inspite of the treatment with heparin. We report with autopsy and literatual reviews.
Autopsy ; Child, Preschool ; Heparin ; Humans ; Male ; Measles ; Purpura Fulminans* ; Purpura*

No abstract available.
Plasma ; Protein C Deficiency* ; Protein C* ; Purpura Fulminans* ; Purpura* ; Warfarin

Purpura fulminans(PF) is a devastating disorder characterized by rapidly progressing hemorrhagic necrosis of the skin, circulatory collapse, and disseminated intravascular coagulation. Histopathologically skin lesions show thrombi within the dermal vessels, a sparse perivascular mononuclear infiltrate, and epidermal necrosis. PF can be classified into three distinct categories: acute infectious PF (sepsis associated PF), hemostasis induced PF, and idiopathic PF. We describe a case of acute infectious PF occurring in 65-year old female with pneumococcal sepsis.
Aged ; Disseminated Intravascular Coagulation ; Female ; Hemostasis ; Humans ; Necrosis ; Purpura Fulminans* ; Purpura* ; Sepsis ; Shock ; Skin

Meningococcal sepsis-associated purpura fulminans is a rapidly progressing condition with high morbidity and mortality. There are several reports of amputation of extremities due to gangrenous change in this condition. However, in Korean literature, we found only one case report associated with amputation of one leg due to meningococcal infection. We report a case of meningococcal infection necessitating the amputation of both legs in a previously healthy seven-year-old girl with a review of literatures.
Amputation* ; Child* ; Extremities ; Female ; Humans ; Leg* ; Meningococcal Infections* ; Mortality ; Purpura Fulminans

Meningococcal sepsis-associated purpura fulminans is a rapidly progressing condition with high morbidity and mortality. There are several reports of amputation of extremities due to gangrenous change in this condition. However, in Korean literature, we found only one case report associated with amputation of one leg due to meningococcal infection. We report a case of meningococcal infection necessitating the amputation of both legs in a previously healthy seven-year-old girl with a review of literatures.
Amputation* ; Child* ; Extremities ; Female ; Humans ; Leg* ; Meningococcal Infections* ; Mortality ; Purpura Fulminans

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.
Hemoglobinuria, Paroxysmal ; Hemolysis ; Humans ; Male ; Middle Aged ; Purpura Fulminans ; Thrombophilia ; Thrombosis

Purpura fulminans is a term that describes an acute, often lethal, syndrome of hemorrhagic necrosis of the skin, due to dermal vascular thrombosis associated with vascular collapse and disseminated intravascular coagulation. Although it is seen in several clinical settings, it occurs most commonly in patients with acute, current infection. The causative organisms include meningococci, Gram-negative bacilli, staphylococci, streptococci and rickettsia. We report a case of streptococcal toxic shock syndrome in a 63-year-old female. She presented with irregular-shaped, massive ecchymoses and hemorrhagic bullae with progressive skin necrosis on her left thigh. Streptococcus pyogenes was found from blood culture and bulla, and multiple organ dysfunction occurred.
Disseminated Intravascular Coagulation ; Ecchymosis ; Female ; Humans ; Middle Aged ; Necrosis ; Purpura Fulminans* ; Purpura* ; Rickettsia ; Shock, Septic* ; Skin ; Streptococcus pyogenes ; Thigh ; Thrombosis

Purpura fuIminans is a rare form of nonthrombocytapenic purpura characterized by sudden onset, fever, prostration, anemia and symmetrical massive ecchymoses, usually of the lower extremities, and hypotension associated with disseminated intravascular coagulation (DIC). A 18 years old female patieat with characteristic clinical manifestations of purpura fulminans was seen at the department of Dermatology, St. Pauls Hospital. With intensive systemic corticasteroid and heparin treatmemts, the ecchymoses and necrosis began to heal, leaving multiple, thick esehar formation from 8th day, Although purpura fulminana has been known to attack mainly children, this case was of adolescent age.
Adolescent ; Anemia ; Child ; Dermatology ; Disseminated Intravascular Coagulation ; Ecchymosis ; Female ; Fever ; Heparin ; Humans ; Hypotension ; Lower Extremity ; Necrosis ; Purpura Fulminans* ; Purpura*

Homozygous Protein C deficiency is a rare genetic disease with catastrophic and fatal purpura fulminans like or thrombotic complication occurring during the neonatal period. Purpura fulminans is characterized by microvascular thrombosis in the dermis followed by perivascular hemorrhage, necrosis, and minimal inflammation. Laboratory findings are consistent with disseminated intravascular coagulopathy: We report a case of purpura fulminans in a neonate with the findings of disseminated intravascular coagulopathy and an undetectable level of protein C activity, whose parents proved to be heterozygous protein C deficiency.
Dermis ; Hemorrhage ; Humans ; Infant, Newborn ; Inflammation ; Necrosis ; Parents ; Protein C Deficiency* ; Protein C* ; Purpura Fulminans* ; Purpura* ; Thrombosis