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

Protein C (PROC) is a potent anticoagulant inactivating coagulation factors Va and VIIIa. PROC deficiency is very rare condition inherited as an autosomal dominant or recessive trait, and associated with various thromboembolic and ischemic conditions. Moreover, severe form of PROC deficiency can cause fatal hemorrhagic complications due to consumptive coagulopathy. We reported two children with hemorrhagic stroke who were diagnosed as severe PROC deficiency caused by two different types of compound heterozygous PROC gene mutations. We described results of laboratory tests, genetic analysis, brain magnetic resonance images, and functional outcomes. Both children received prophylactic anticoagulation therapy and presented with purple-colored skin lesions during rehabilitation. Purpura fulminans caused by insufficient anticoagulation should be differentiated from hematoma caused by excessive anticoagulation therapy in these children.
Blood Coagulation Factors ; Brain ; Cerebral Palsy* ; Child ; Hematoma ; Humans ; Intracranial Hemorrhages* ; Protein C Deficiency* ; Protein C* ; Purpura Fulminans ; Rehabilitation ; Skin ; Stroke

Protein C (PROC) is a potent anticoagulant inactivating coagulation factors Va and VIIIa. PROC deficiency is very rare condition inherited as an autosomal dominant or recessive trait, and associated with various thromboembolic and ischemic conditions. Moreover, severe form of PROC deficiency can cause fatal hemorrhagic complications due to consumptive coagulopathy. We reported two children with hemorrhagic stroke who were diagnosed as severe PROC deficiency caused by two different types of compound heterozygous PROC gene mutations. We described results of laboratory tests, genetic analysis, brain magnetic resonance images, and functional outcomes. Both children received prophylactic anticoagulation therapy and presented with purple-colored skin lesions during rehabilitation. Purpura fulminans caused by insufficient anticoagulation should be differentiated from hematoma caused by excessive anticoagulation therapy in these children.
Blood Coagulation Factors ; Brain ; Cerebral Palsy* ; Child ; Hematoma ; Humans ; Intracranial Hemorrhages* ; Protein C Deficiency* ; Protein C* ; Purpura Fulminans ; Rehabilitation ; Skin ; Stroke

Protein C (PROC) deficiency is caused by mutations in the PROC gene on chromosome 2q14.3. Patients with PROC deficiency typically present distinguished purpura, intracerebral and intravascular coagulopathy, and ophthalmologic complications. Here, we report a rare severe form of PROC deficiency resulting from a compound heterozygosity in PROC. The patient was a 5-day-old female neonate born at 39 weeks of gestation with a birth weight of 2,960 g. She was transferred to our hospital with running a fever at 38.5℃ and with dark red patches on her feet. At admission, a complete blood count showed no specific findings, but levels of PROC and protein S were abnormally low (1% and 68%, respectively). Magnetic resonance imaging revealed intracerebral hemorrhaging and parenchymal damage with dysplasia of the brain. Ophthalmologic examination revealed vitreous hemorrhaging with retinal detachment. Genetic testing revealed a missense mutation (Arg211Trp) and a frameshift mutation (Gly239Serfs*8) in PROC, inherited from the father and mother, respectively. The patient recovered from purpura after undergoing ventriculoperitoneal shunting and treatment with fresh frozen plasma, warfarin sodium, and PROC concentrate. This is the first report of severe neonatal PROC deficiency with purpura fulminans, vitreous hemorrhage, and intracerebral hemorrhage confirmed via PROC genetic testing, which identified a rare compound heterozygosity of PROC.
Birth Weight ; Blood Cell Count ; Brain ; Cerebral Hemorrhage ; Diagnosis* ; Fathers ; Female ; Fever ; Foot ; Frameshift Mutation ; Genetic Testing ; Humans ; Infant, Newborn ; Magnetic Resonance Imaging ; Mothers ; Mutation, Missense ; Plasma ; Pregnancy ; Protein C Deficiency* ; Protein C* ; Protein S ; Purpura ; Purpura Fulminans ; Retinal Detachment ; Running ; Ventriculoperitoneal Shunt ; Vitreous Hemorrhage ; Warfarin

PURPOSE: Purpura fulminans is a rare but rapidly progressive, serious, often life-threatening disorder in childhood, which is complicated with septic shock or disseminated intravascular coagulopathy during acute infection. It occurs first as acute-onset petechial rash, and spreads rapidly into full thickness skin and soft tissue necrosis. In the past, it had high mortality rate, up to 80%, but recently, survival rate has increased due to early diagnosis, and rapid advancement of critical care and antibiotics. From our experiences of PF management, we would like to review the pathophysiology and suggest the surgical treatment guideline about meningococcal induced purpura fulminans. METHODS: Two cases of purpura fulminans over the last 3 years were reviewed retrospectively about reconstructive management. After they were treated resuscitative management initially by the critical intensive care, reconstructive surgery was performed by plastic surgeon as soon as the patients were vitally and mentally stable. RESULTS: There were 6 procedures in case 1, and 3 procedures in case 2. The mean delayed period from admission with sepsis to the first surgical debridement was 24 days and 42 days, respectively. Total hospitalization period was 103 days and 69 days, respectively. All of them were treated with debridement and split thickness skin graft, but delayed debridement was superior to early one in the point of preserving much more tissues. CONCLUSION: From our experience, we suggest that conservative therapy to the wounds appears to be the best tool in the initial vitally unstable period in order to preserve as much tissues and functions as possible if no active inflammation and compartment syndrome are detective.
Anti-Bacterial Agents ; Compartment Syndromes ; Critical Care ; Debridement ; Early Diagnosis ; Exanthema ; Hospitalization ; Humans ; Inflammation ; Critical Care ; Mortality ; Necrosis ; Purpura Fulminans* ; Purpura* ; Retrospective Studies ; Sepsis ; Shock, Septic ; Skin ; Survival Rate ; Transplants ; Wounds and Injuries

PURPOSE: Purpura fulminans is a rare but rapidly progressive, serious, often life-threatening disorder in childhood, which is complicated with septic shock or disseminated intravascular coagulopathy during acute infection. It occurs first as acute-onset petechial rash, and spreads rapidly into full thickness skin and soft tissue necrosis. In the past, it had high mortality rate, up to 80%, but recently, survival rate has increased due to early diagnosis, and rapid advancement of critical care and antibiotics. From our experiences of PF management, we would like to review the pathophysiology and suggest the surgical treatment guideline about meningococcal induced purpura fulminans. METHODS: Two cases of purpura fulminans over the last 3 years were reviewed retrospectively about reconstructive management. After they were treated resuscitative management initially by the critical intensive care, reconstructive surgery was performed by plastic surgeon as soon as the patients were vitally and mentally stable. RESULTS: There were 6 procedures in case 1, and 3 procedures in case 2. The mean delayed period from admission with sepsis to the first surgical debridement was 24 days and 42 days, respectively. Total hospitalization period was 103 days and 69 days, respectively. All of them were treated with debridement and split thickness skin graft, but delayed debridement was superior to early one in the point of preserving much more tissues. CONCLUSION: From our experience, we suggest that conservative therapy to the wounds appears to be the best tool in the initial vitally unstable period in order to preserve as much tissues and functions as possible if no active inflammation and compartment syndrome are detective.
Anti-Bacterial Agents ; Compartment Syndromes ; Critical Care ; Debridement ; Early Diagnosis ; Exanthema ; Hospitalization ; Humans ; Inflammation ; Critical Care ; Mortality ; Necrosis ; Purpura Fulminans* ; Purpura* ; Retrospective Studies ; Sepsis ; Shock, Septic ; Skin ; Survival Rate ; Transplants ; Wounds and Injuries

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

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

The most serious problem after splenectomy is the increased risk of life-threatening infections caused by encapsulated bacteria such as Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis. Purpura fulminans, which is commonly associated with meningococcal sepsis, is characterized by disseminated intravascular coagulation and rapidly progressive purpuric skin lesions. Purpura fulminans can also develop in invasive pneumococcal infection especially after splenectomy, however, there has been no report in Korea. We report a case of overwhelming pneumococcal sepsis manifested as purpura fulminans in a splenectomized patient.
Bacteria ; Disseminated Intravascular Coagulation ; Haemophilus influenzae ; Humans ; Korea ; Neisseria meningitidis ; Pneumococcal Infections ; Purpura Fulminans* ; Purpura* ; Sepsis ; Skin ; Splenectomy ; Streptococcus pneumoniae* ; Streptococcus*