Blue Rubber Bleb Nevus Syndrome: Promising Response To Sirolimus.
نویسندگان
چکیده
BACKGROUND Blue rubber bleb nevus syndrome is a rare disease involving venous malformations. CASE CHARACTERISTICS We present a 6-year-old female with the syndrome, and consumptive coagulopathy. INTERVENTION/OUTCOME After management with sirolimus, symptoms resolved. MESSAGE Sirolimus may be a valuable option for reducing bleeding complications and cosmetic sequelae for the patients with this syndrome.
منابع مشابه
Extensive Blue Rubber Bleb Nevus Syndrome with Multiple Gastrointestinal Venous Malformations: A Case Report
Blue rubber bleb nevus syndrome is a sporadic disease with widely distributed dark blue papules and nodules and soft skin-colored compressible protuberances (rubber blebs) as well as large vascular malformations. Gasterointestinal lesions are documented by upper endoscopy and colonoscopy; hemorrhages from these lesions create irondeficinecy anemia. Other sites of visceral involvement are less c...
متن کاملThe management of ophthalmic involvement in blue rubber bleb nevus syndrome
OBJECTIVE Blue rubber bleb nevus syndrome is a rare vascular disease most commonly associated with venous malformations of the skin and the gastrointestinal tract. Few ophthalmic cases have been reported to date, and no clear treatment regimen exists. We describe the case of a 59-year-old man, along with a review of literature, to help in the future diagnosis and treatment of patients with the ...
متن کاملBlue rubber bleb nevus syndrome: successful treatment with sirolimus.
Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder with characteristic vascular malformations of the skin, gastrointestinal system, and, less often, other organ systems. The characteristic cutaneous lesions consist of deep-blue, soft, rubbery blebs, which are easily compressible. The most serious complication is abundant gastrointestinal bleeding. We describe the case of an 8-year-old g...
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RATIONALE Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterized by multiple venous malformations. The gastrointestinal bleeding and secondary iron deficiency anemia are the most common complications. There are currently no effective treatments for BRBNS. Here, we report a case of successful treatment with a small dose of sirolimus of a BRBN patient with a de novo gene mutation...
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The Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare cause of persisting gastrointestinal bleeding. We are presenting a case which was associated with an atrial septal defect.
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ورودعنوان ژورنال:
- Indian pediatrics
دوره 54 1 شماره
صفحات -
تاریخ انتشار 2017