Multiple Skin and Gastro-intestinal Haemangiomata (Blue Rubber-Bleb Nevus)

Abstract

A case of the so-called blue rubber-bleb nevus, which appeared in old age, with an anorectal bleeding is presented. Thrombocytopenia and hypercalcemia present in the case, are discussed and the pertinent literature is reviewed. The curious coincidence of 2 cystic squamous cell carcinoma in the groin is discussed, in view of the reported coincidence of other tumours. We propose to call this entity ‘multiple skin and gastro-intestinal haemangiomata’. This because the name describes the main features of this syndrome; moreover, it would call attention to the possibility of gastro-intestinal haemangiomata in such cases.

Blue rubber-bleb naevus syndrome arising in the middle age

“…Thus, in order to rule out the presence of potentially threatening lesions (particularly in the small bowel), the execution of the overmentioned laboratory screening is necessary even in patients with a mild and/or late onset and with a history unrevealing for past haemorrhagic events. By the way, the gastrointestinal involvement was always present in the few cases of adult‐onset BRBNS previously reported in literature and supported by explicit anamnestic data (10,29,30).…”

Blue Rubber Bleb Nevus Syndrome

“…A few cases of BRBN syndrome (24-27) started in adulthood; the oldest reported patient was 65 years old when hemangiomas developed (24). Other ages of onset were about 63 years (25), 19 years (26), and unknown (less than 46 years) (27).…”

“…Other ages of onset were about 63 years (25), 19 years (26), and unknown (less than 46 years) (27). One woman, age 75 years, suffered from hemangiomas for about 10 years (24). Family history was negative for this disorder.…”