A Case of Blue Rubber Bleb Nevus Syndrome With Kasabach-Merritt Syndrome and Heart Failure

Abstract

A 48-year-old Mongolian man developed bilateral leg edema after suffering from a fever for three months. He lost his appetite, and the edema gradually spread from the legs, becoming systemic. In addition, he had difficulty in moving. He had a history of being diagnosed with numerous venous malformations and Kasabach-Merritt syndrome when he was a child. On arrival, he had numerous venous malformations over pale skin, edema at each extremity, and anemic conjunctiva. Chest roentgen showed bilateral pleural effusion, and cardiac echography findings showed a left ventricular ejection fraction of 30% with diffuse hypokinesis. The results of a blood analysis showed coagulopathy, which was compatible with disseminated intravascular coagulation and pancytopenia. He was diagnosed with blue rubber bleb nevus syndrome with Kasabach-Merritt syndrome and heart failure. Use of diuretics, thiamine, iron, phytonadione, carbazochrome, and tranexamic acid, in addition to intermittent transfusion resulted in the improvement of his Kasabach-Merritt syndrome. Radical management of blue rubber bleb nevus syndrome was deemed impossible by dermatologists due to the large amount of venous malformations. We encountered an extremely rare case of blue rubber bleb nevus syndrome with Kasabach-Merritt and heart failure. Multimodal therapy might help manage Kasabach-Merritt syndrome following improvement in coagulopathy and pancytopenia.

Case report: Blue rubber bleb nevus syndrome with Kasabach-Merritt phenomenon in a neonate

“…The occurrence of BRBNS with KMP is extremely rare. Jitsuiki et al (7) reported a similar case in a Japanese study. At present, no similar report from China has been found.…”