An 80-year-old leukodermic man presented with iron deficiency anemia (hemoglobin 10.4 g/dL; mean corpuscular volume (MCV) 75 fL; serum ferritin 15 ng/mL) His past history included arterial hypertension and atrial fibrillation. Medication history included dabigatran. The patient had no history of non-steroidal anti-inflammatory drug use, peptic ulcers, or chronic liver disease. No relevant family history was recorded. He denied having recurrent epistaxis or overt gastrointestinal bleeding. He underwent an esophago-gastroduodenoscopy and colonoscopy as part of the workup for iron deficiency anemia; these were unrevealing. He then underwent capsule endoscopy; multiple, protruding, nodular, bluish lesions were found in the small bowel (Fig. 1, 2) without active bleeding. Given the clinical and endoscopic features, his medical records were reviewed, and a previous dermatology appointment was found where multiple, violaceous, compressible, nonpulsatile nodular lesions were described on the skin (Fig. 3). Altogether, given the multifocal, venous vascular malformations, we established a diagnosis of blue rubber bleb nevus syndrome (BRBNS). The patient is currently on oral iron supplementation with a good clinical response (hemoglobin 12.4 g/dL)

Fig. 1: Multiple protruding, nodular, bluish lesions throughout the small bowel. 

Fig. 2: Multiple protruding, nodular, bluish lesions throughout the small bowel. 

Fig. 3: Multiple violaceous, compressible, and nonpulsatile nodular cutaneous lesions. 

BRBNS (or Bean’s disease) is a rare disease mainly characterized by multiple venous malformations that can affect any organ system, but frequently affecting the cutaneous and gastrointestinal systems (¹-³). The etiology and pathogenesis remain uncertain (¹, ³). The majority of cases are sporadic, although autosomal inheritance has been identified associated with the chromosome 9p (¹, ²). The diagnosis is based on the presence of characteristic venous malformations, and up to 87% of patients have multiple organ involvement (¹);. Lesions are often present from birth or may develop during childhood; however, 4% of cases present during adulthood (¹). The cutaneous lesions are characteristically rubbery, soft, and easily compressible (they promptly refill after compression) (¹, ²). Gastrointestinal lesions are mostly found in the small bowel and distal large bowel, and are typically bluish nodules (¹, ²). Individuals with gastrointestinal involvement typically present with gastrointestinal bleeding or iron deficiency anemia (¹, ², ⁴). The treatment of this syndrome is usually conservative and should be guided by the topography of the vascular lesions and disease severity (¹, ², ⁴). Accordingly, if needed, gastrointestinal lesions can be managed with endoscopic therapy or surgery (¹, ², ⁴). Cutaneous lesions, on the other hand, generally do not require treatment (¹). Finally, systemic medical treatment with antiangiogenic agents like sirolimus have been successfully used as a rescue treatment (¹, ⁵). In this particular case, the beginning of anticoagulation might have been the key for the diagnosis of this late-onset BRBNS since it might have triggered a common manifestation of BRBNS. We emphasize the utmost importance of considering the full medical history as well as a physical examination, in order to provide an adequate endoscopic diagnosis, especially when considering systemic disorders involving the gastrointestinal tract.