RESUMEN
BACKGROUND Vascular Behçet's disease (VBD) is a rare but potentially life-threatening subtype of Behçet's disease that is characterized by multisystemic vasculitis. It primarily affects males with ancestry traced back to regions along the ancient Silk Road. Both arteries and veins, regardless of size, may exhibit complications, including aneurysmal degeneration or occlusion. While venous involvement is observed in two-thirds of VBD cases, arterial complications are notably the most severe and lethal. Arterial aneurysmal degeneration is more common than occlusive complications, with larger arteries being predominantly affected in VBD. Data regarding isolated small-vessel arterial occlusive disease in VBD are limited. Given the rarity of this presentation in this patient population, it becomes mandatory to thoroughly evaluate such patients to differentiate small-vessel vasculitis from other similar diseases, such as Raynaud's phenomenon, which has a different etiology and management and generally has a more benign course. Here, we delineate the concept of isolated small-vessel vasculitis as a cause of blue toe syndrome in patients with VBD. CASE REPORT This report describes a distinctive case of vascular Behçet's disease in a 51-year-old man who initially exhibited unilateral blue toe syndrome, which swiftly progressed to dry gangrene of the toes. Despite reports of large-vessel involvement, there is a paucity of data on isolated small-vessel vasculitis-induced digital ischemia in VBD. CONCLUSIONS This atypical case underscores the necessity of clinical discernment in differentiating inflammatory microvascular occlusive disease from vasospastic Raynaud's syndrome, both of which can complicate Behçet's disease.
Asunto(s)
Síndrome de Behçet , Síndrome del Dedo Azul , Humanos , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico , Masculino , Persona de Mediana Edad , Síndrome del Dedo Azul/etiologíaRESUMEN
BACKGROUND Splenic hamartoma (SH) is a benign vascular lesion, usually found incidentally on abdominal images or at autopsy. Only around 200 cases have been reported since 1861, when SH was first described by Rokitansky. Although it is very rare, it is important to be familiar with it, as it may be a diagnostic challenge to distinguish SH from other mass lesions of the spleen based solely on preoperative investigations. CASE REPORT We describe a case of symptomatic, isolated, single splenic hamartoma in a 19-year-old, otherwise healthy young man who presented with upper abdominal pain, nausea, and vomiting for a few months. The examination was unremarkable. The patient has been previously evaluated with abdominal ultrasonography, which found a suspicious splenic hyperechoic lesion. Computed tomography revealed a heterogeneous 5×7 cm enhancing lesion in the spleen, concerning for splenic hamartoma. The patient underwent laparoscopic splenectomy and recovered well. The histopathology examination confirmed the diagnosis of splenic hamartoma. CONCLUSIONS Splenic hamartoma is a rare benign vascular lesion of debated etiology. Most cases are asymptomatic and are found incidentally on images, in splenectomies performed for other reasons, or at autopsy. Radiologic findings may suggest the diagnosis and new modalities have shown accuracy in distinguishing splenic hamartomas. However, resection with formal or partial splenectomy is usually still needed since the differential diagnosis is wide, from benign to aggressive lesions, and histopathology remains the criterion standard for diagnosis. Given its benign nature, we found no cases of recurrence or metastasis in the literature.