ABSTRACT
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a rare genetic disorder originating from a somatic mutation in the hematopoietic stem cells. This syndrome was first described in 2020 and carries many clinical features that other conditions cannot explain. Widespread autoinflammation is the primary process the disease presents, with high morbidity and mortality in those who show signs of bone marrow failure. Treatment is complex, and response to current therapies is poor. Long-term prognosis carries a mortality of 50%. In addition, the advancement of new-generation messenger ribonucleic acid (mRNA) vaccines raises concerns about their safety in this population since it could trigger a vaccine-related autoimmune response. This case describes the hospital course of a male in his 50s exhibiting an unexplained cutaneous reaction to an mRNA COVID-19 vaccine. He was later diagnosed with VEXAS syndrome based on symptoms presentation and diagnostic workup.
ABSTRACT
Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is a novel coronavirus that has been extensively described in its most common presentations. Its pathogenetic process is poorly understood, although it is theorized that endothelial damage and inflammation play a central role. Its prothrombotic nature affects multiple organs, including lungs, kidneys, and the central nervous system. Rarer are cutaneous presentations that can be triggered or displayed concomitantly with COVID-19. Purpura fulminans is a life-threatening syndrome that results in skin thrombosis and hemorrhagic infarction. While its association is explicit in critically ill patients with sepsis, few or rare cases have been described to be linked with COVID-19. In this report, we present a case of a critically ill patient with COVID-19 who showed signs of purpura fulminans while in the intensive care unit.