Síndrome de Bean.

BACKGROUND: Bean syndrome is a rare congenital disorder associated with autosomal dominant inheritance, and mutation of chromosome 9p. It is characterized by multiple recurrent vascular malformations. CLINICAL CASE: A 14-year-old female patient who presented with intermittent headache, tachycardia and fatigue in daily activities. On physical examination, there were nodular violaceous raised lesions on the tongue, approximately 1 x 1 cm, non-bleeding. Additionally, disseminated red-violaceous nodular lesions were observed on the upper and lower limbs. In the endoscopic evaluation, there was evidence of multiple 2 to 8 mm red-violet elevated lesions from the esophago-gastric junction, stomach, to the second duodenal portion. The MRI showed solid tissue located to the left of the brain sickle at the level of the ipsilateral parietal lobe. The diagnosis was Bean syndrome associated with idiopathic thrombocytopenic purpura. Symptomatic treatment, blood transfusion and immunosuppressive management with sirolimus were provided. The evolution was favorable during admission and subsequent hospital admissions. CONCLUSIONS: The timely diagnosis of Bean syndrome is important for its adequate therapeutic management, due to its isolated presentation, considering a key factor the evaluation in young patients who present multiple skin lesions and gastrointestinal tract involvement that later leads to blood transfusions. [ABSTRACT FROM AUTHOR]