An Unusual Case of Adult-Onset Still’s Disease with Hemophagocytic Syndrome, Necrotic Leukoencephalopathy and Disseminated Intravascular Coagulation

Case. A 34-year-old African-American female with a history of adult-onset Still’s disease presented to an outside hospital with oligoarthritis. She experienced a generalized tonic-clonic seizure en route via ambulance, was intubated upon arrival, and transferred to the intensive care unit for treatment of suspected pneumonia and sepsis. She subsequently developed generalized cutaneous desquamation that progressed despite the cessation of antibiotics and other potential offending drugs which required transfer to our hospital’s burn unit. She was suspected to have reactive hemophagocytic syndrome based on her clinical presentation of fever, rash, polyarthritis, elevated liver enzymes, coagulopathy, splenomegaly, normocytic anemia, thrombocytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis visualized in bone marrow biopsy specimen. Magnetic resonance imaging demonstrated necrotic demyelination of the deep white matter and corona radiata. The patient developed multiorgan dysfunction and DIC without any other attributable etiology. Despite aggressive broad spectrum therapy and high dose of steroids she progressively deteriorated and eventually expired. Conclusion. Previous publications have highlighted the prevalence of necrotic leukoencephalopathy in children with familial hemophagocytic syndrome. Our patient demonstrated some uncommon features complicating her HLH including DIC and necrotic leukoencephalopathy, which are very rare entities in AOSD. 1. Introduction Still’s disease was first described by George Still in 1896. Adult-onset Still’s disease (AOSD) describes a similar condition which affects adults and is generally constituted by recurrent high grade fever with concomitant salmon-colored rash of the trunk and extremities, oligo- and later polyarthritis, pharyngitis, splenomegaly, lymphadenopathy, and less commonly pericarditis or pleural effusions [1]. Laboratory evaluation typically demonstrates elevated erythrocyte sedimentation rate, leukocytosis, and elevated liver enzymes [2]. Hemophagocytic syndrome (HS), also termed hemophagocytic lymphohistiocytosis (HLH), is a syndrome of immune activation manifested by signs and symptoms of inflammation [3]. HS is diagnosed based on the fulfillment of 5/8 criteria as in Table 1 [4]. Histopathological evaluation demonstrates lymphocytes, macrophages and hemophagocytosis in bone marrow, spleen, cerebral spinal fluid, and liver [4]. Other common clinical manifestations include central nervous system involvement, coagulopathy, liver dysfunction, jaundice, edema, and skin rashes [4]. A