Parvovirus B19-associated catastrophic endothelialitis with a Degos-like presentation
Malignant Atrophic Papulosis
Parvovirus B19 is a single-stranded DNA virus exhibiting affinity for a variety of cell types including endothelial cells. The basis of the affinity is globoside expression, the receptor for B19. B19 endothelial cell parasitism accelerates endothelial cell apoptosis, potentially critical in inducing neoantigenicity and secondary antibody formation. A 38-year-old previously healthy male developed intestinal and cutaneous infarction, elevated creatinine phosphokinase (CPK) levels and progressive cytopenias. The basis of his clinical presentation was unclear until a skin biopsy suggested a potential role for virally mediated endothelial cell injury. Hematoxylin and eosin, immunohistochemical, immunofluorescent and reverse transcriptase in situ polymerase chain reaction studies were conducted on skin biopsies to assess for viral triggers. B19 viremia and localization of B19 RNA transcripts to vascular endothelium were uncovered. A diagnosis of catastrophic endothelial cell injury attributable to B19 infection was rendered. The patient showed significant improvement with intravenous immunoglobulin (IVIG). Parvovirus B19 is a ubiquitous virus that in the majority of affected patients remains asymptomatic. Nevertheless, because of its ability to infect endothelium it can cause severe multiorgan endothelial cell injury.