Case Report
 
Littoral cell angioma in a patient with Ehlers-Danlos syndrome on biologic immunosuppression for psoriatic arthritis
Daniel Gorman, MS, OMS41,2, Reece Bergstrom, OMS41,2
1OMS-4, Touro College of Osteopathic Medicine, New York, NY.
2OMS-4, Englewood Hospital & Medical Center, Englewood, NJ.

Article ID: Z01201612CR10727DG
doi:10.5348/ijcri-2016139-CR-10727

Address correspondence to:
Reece Bergstrom
Englewood Hospital & Medical Center
Englewood
NJ

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How to cite this article
Gorman D, Bergstrom R. Littoral cell angioma in a patient with Ehlers-Danlos syndrome on biologic immunosuppression for psoriatic arthritis. Int J Case Rep Images 2016;7(12):800–804.


Abstract
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 TNFα 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.

Keywords: Hemangioma, Littoral cell angioma, Vascular tumors


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Author Contributions
Daniel Gorman – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Reece Bergstrom – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Daniel Gorman et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.