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Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital disease, characterized by the presence of multiple venous malformations in the skin, gastrointestinal tract and other organs. Small bowel (SB) complications are intussusception, volvulation, and small bowel bleeding (SBB).1 Surgery may be limited by the multiplicity of lesions, morbidity, and need for intraoperative endoscopy.2 Although close follow up is mandatory because of the risk of recurrence, endoscopy may be an alternative.3 A 21‐year‐old woman diagnosed with BRBNS presented at Cabueñes University Hospital with iron deficiency anemia (hemoglobin: 6 g/dL) and continuous transfusion requirements. Three venous malformations located in the stomach and colon were treated by conventional endoscopy. SBB was suspected because of persistent iron deficiency anemia. Therefore, capsule endoscopy was carried out, detecting three polypoid angiomatous lesions in the middle jejunum. Four soft, sessile venous malformations of 1–2 cm were confirmed by oral double‐balloon enteroscopy (DBE) under general anesthesia (Fig. 1).

Vascular malformations are errors of development of vessels that occur during embryogenesis. They are rheologically divided into slow-flow (capillary, lymphatic, venous and combined) and fast-flow (arterial, arteriovenous, and combined) malformations. These congenital lesions grow proportionally with the patient and never spontaneously regress. Venous malformations (VM) are composed of ectatic venous-like channels. They are mainly sporadic but can be inherited as an autosomal pattern. Any tissue and organ can be affected. They can be isolated or part of syndromes. Blue rubber bleb naevus syndrome is characterized by multifocal rubbery cutaneous venous malformations, especially on palm and sole, that are associated with multiple gastrointestinal VM. Localized intravascular coagulopathy, with high D-dimer level and normal-to-low fibrinogen level, is commonly seen. As cerebral VM can also occur in this syndrome, brain MRI should be part of the screening of patients affected with BRBN. Management is often multidisciplinary and can include medical, interventional radiological and/or surgical treatment.

Blue nevi are congenital or acquired, dermal dendritic melanocytic proliferations that can simulate melanocytic and nonmelanocytic lesions including melanoma, cutaneous metastasis of melanoma, Spitz/Reed nevi, and basal cell carcinoma.

Venous malformations (VM) are localized defects of blood vessels that are due to vascular dysmorphogenesis. These slow-flow lesions can affect any tissue or organ. Clinically, a cutaneous VM is characterized by a bluish mass that is compressible on palpation. Phleboliths are commonly present. Symptoms depend on location and size. VM are often sporadic and isolated, however, they can be associated with other malformations and be part of a syndrome; Klippel-Trenaunay (capillary-lymphatico-venous malformation with limb hypertrophy) is the most common. Glomuvenous malformation (GVM) is another type of venous anomaly. In contrast to VM, GVM is often painful on palpation and not compressible. Clinical diagnosis of VM is often made in the presence of a bluish cutaneous lesion: however, other lesions can mimick VM. The most frequent anomalies are a blue naevus, a hemorrhagic lymphatic malformation, a sub-cutaneous hemangioma or even the presence of dilated superficial normal veins due to underlying venous stenoses. This chapter will detail the clinical characteristics of venous anomalies and their differential diagnosis.

The combined nevus is a clinical simulator of melanoma, and clinical examination alone can be inadequate to diagnose this melanocytic pigmented skin lesion. Dermoscopy is a useful tool to differentiate the features related to each type of nevus. We report two cases of combined nevi dermoscopically characterized by a diffuse blue-whitish pigmentation similar to the blue-whitish veil, suggesting the diagnosis of melanoma.

A combined nevus most commonly consists of a blue nevus in combination with a Clark or Spitz nevus. Dermoscopically, combined nevus can mimic melanoma owing to the presence of dermoscopic features common to both types of lesions. Benign clinical and dermoscopic changes can occur in nevi over time, especially in children and young adults.