[Blue-rubber-bleb-nevus syndrome presented vascular dementia and chronic DIC--a case report].

We reported a female with vascular dementia due to chronic disseminated intravascular coagulation (DIC). The patient underwent operation for skin hemangiomas three times at age 18, 27, and 38. At age 43, she presented a slowly progressive dementia. She had bluish, compressive hemangiomas of skin and upper gastrointestinal tract, and she was suspected to have hemangiomas of brain, pleura, mediastinum, liver and pancreas. The hemangiomas were diagnosed blue-rubber-bleb-nevus syndrome (BRBNS). Laboratory data revealed persistent increase of serum FDP, FDP-D, FDP-E, and D-dimer. This condition was considered chronic DIC, therefore this case was the first case of combination of BRBNS and Kasabach-Merritt syndrome (chronic DIC). Brain MRI demonstrated bilateral lesions of paraventricular deep white matter and hemangioma of left temporal lobe. Because cerebral CT showed no contrast enhancement in paraventricular lesions, the lesions were considered multiple cerebral infarctions. Central nervous system (CNS)involvement in BRBNS is rare, and most cases of CNS involvement were associated with CNS angiomata. In this case it was suggested that brain was the target organ of DIC because thrombomodulin is absent or few in the human brain, therefore vascular dementia (multiple cerebral infarctions) developed due to chronic DIC.