Leopard Can't Change Its Spots - Presentation PPT

Bone marrow biopsy showed markedly increased histiocytes with increased erythrophagocytosis, platelet phagocytosis, and rare hemophagocytosis, consistent with a diagnosis of HLH. The close temporal relationship between panuveitis and TEN, two inflammatory events with similar skin presentations, together with immunological findings of positive parvovirus IgG, and bone marrow parvovirus PCR indicative of a chronic type of infection, supported a chronic parvovirus infection. Although rare, HLH has been described as having uveitis as a symptom of the disease. 1 It is unclear why there was a delay between the eye findings and the systemic features of her illness. Parvovirus B19 has been described as the inciting factor in uveitis and HLH. 2,3 Perhaps in our case there was a delayed activation of the immune system to parvovirus that initially incited inflammation in the eye and then progressed to a systemic inflammatory response, as shown by the elevated CRP. As her condition progressed from eye inflammation, she developed encephalopathy with MRI brain findings of bilateral thalamic signal intensities, which can be associated with viral infections. Along with her characteristic lab findings, the bone marrow pathology was critical in showing hemophagocytosis to help make the diagnosis. This case helps physicians remember to keep the rare diagnosis of HLH in mind when there are multiple systemic manifestations in conjunction with eye inflammation that all point to a possible autoimmune etiology. This patient was treated with systemic steroids along with aggressive fluid, airway, and burn management. Her symptoms gradually improved along with the recovery from critical status.