Littoral cell angioma in a patient with Ehlers–Danlos syndrome on biologic immunosuppression for psoriatic arthritis

Littoral cell angioma (LCA) of the spleen is a rare, benign vascular tumor first described in 1991. The etiology and pathogenesis continues to be unclear. Only a few dozen cases have been noted since its first original identification. This case report discusses a patient in which hypodense splenic lesions were incidentally found on computed tomography. The patient elected for a splenectomy and LCA was discovered on pathologic analysis. This case presents an interesting discussion due to the patients’ complex medical history including a previous diagnosis of Ehlers-Danlos syndrome as well as chronic immunosuppression secondary to treatment for psoriatic arthritis. The possible pathogenesis of LCA is unclear. Current theories revolve around either increased or decreased levels of TNFa in disease progression. Altered immune response is linked to LCA, which is confirmed by the resolution of leukocytosis following treatment. Reticuloendothelial system fragility predisposes to structural anomalies such as LCA. Further etiologic studies of patients with this extremely rare vascular tumor with give further indication of the disease pathogenesis.