Bean-Syndrome in Maxillofacial District

Bean syndrome (BS) is a rare congenital angiomatosis affecting multiple organ systems. The exact etiology is still not well-known. About 200 cases with heterogeneous clinical presentations have been reported worldwide. BS predominantly presents with characteristic multifocal mucocutaneous lesions and often leads to iron-deficiency anemia caused by occult blood loss from the gastrointestinal tract. Apart from the mucocutaneous and gastrointestinal tract involvement, this syndrome may rarely affect the craniofacial region in the form of multiple venous anomalies, characterized by being button-like, with a bluish tint, covered by skin, called "Blue Nevus". Patients often approach a dermatologist or gastroenterologist for treatment and this entity is usually less known among maxillofacial surgeons. This article describes two consecutive cases of BS affecting the craniofacial district, with the aim of spreading greater awareness about this syndrome in the maxillofacial field.