Hyper-Immune-Globulin E Syndrome and Cryptococcal Meningitis Leading to a Fatal Outcome in a Young Woman: A Rare and Lethal Combination

Nierenberg RJ, Devasagayaraj R, Nguyen H, Craciun L, Chalabi D, Nguyen H and Guma M

Departments of Emergency Medicine, Neurology, Infectious Disease and Rheumatoolgy, Hackensack University Medical Center, Hackensack Meridian School of Medicine, Nutley, New Jersey, USA

^*Correspondance to: Nierenberg RJ 

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Abstract:

Background: The very rare syndrome, hyper-immunoglobulin E, also known as Job or Buckley syndrome, is characterized by multiple skeletal and cutaneous disorders, but also can present with potentially fatal infectious diseases rarely seen and not always considered. With HIV positive patients, or patients on immunosuppressive agents, for example, Cryptococcal meningitis is often considered for any neurologic presentation; however in patients not appreciated to be immunocompromised consideration of that disorder is frequently delayed with worsening mortality. The Hyper-IgE syndrome has not, to our knowledge, been presented in the emergency medicine literature. Case Report: We present the case of a young woman with this syndrome who presented with neurologic symptoms and in whom the diagnosis of Cryptococcal meningitis was only made after repeated emergency department visits and a several day hospital admission, and who succumbed. Literature showing worse prognosis for CM in non-HIV patients is reviewed, as well as an analysis of cognitive biases in decision making. We believe the introduction of this fortunately exceedingly rare condition of immunocompromised, and review of the too often undetected presentation of CM in non-HIV patients will be of value.

Keywords:

Hyper IgE syndrome; Immunocompromised; Cryptococcal meningitis; Cognitive bias; Rare disease

Cite the Article:

Nierenberg RJ, Devasagayaraj R, Nguyen H, Craciun L, Chalabi D, Nguyen H, et al. Hyper-Immune- Globulin E Syndrome and Cryptococcal Meningitis Leading to a Fatal Outcome in a Young Woman: A Rare and Lethal Combination. Ann Clin Case Rep. 2020; 5: 1903..