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Blue rubber bleb nevus syndrome (BRBNS) is a rare disease that presents as cutaneous and extracutaneous vascular malformations, most commonly affecting the gastrointestinal (GI) tract. We report a case of adult onset BRBNS in an African American male with vascular lesions isolated to the jejunum without any cutaneous manifestations. Physicians should recognize that BRBNS can present without skin involvement and may have complications from visceral organ involvement. Treatment of BRBNS is mainly symptomatic and aims at preserving the GI tract as much as possible. BRBNS may also present as delayed recurrence after surgical or endoscopic interventions.

An unusual and challenging cause of small bowel bleeding: Isolated gastrointestinal blue rubber bleb nevus syndrome