Leopard Can't Change Its Spots - Abstract

Abstract

An 8 year-old Caucasian female presented with bilateral conjunctivitis, photophobia, and blurred vision. Visual acuity was 20/50 OD and 20/60 OS. She had 2-3+ anterior chamber cell and flare OU, 1+ vitreous cells OU, 2+ optic disc edema OU, and macular edema OU. She was diagnosed with anterior uveitis, vitritis, and neuroretinitis. At home, she had a cat, a rabbit, and a dog. She had recent travel to Hawaii. She reported a recent transient erythematous maculopapular rash behind her ears and neck. A full work-up was negative for bartonellosis, brucellosis, leptospirosis, toxoplasmosis, Lyme disease, and tularemia. Normal studies included serum ACE, lysozyme, ANA, chest x-ray, and HLA-B27. Systemic treatment consisted of azithromycin and rifampin. Prednisolone acetate eyedrops were added with a slow taper over 4 months when ocular inflammation resolved and vision returned to 20/20 OU. A week later, she developed new fevers, headache, nausea, vomiting, seizures and altered mental status. She developed disseminated intravascular coagulopathy, and recurrence of maculopapular rash which progressed to toxic epidermal necrolysis (TEN). MRI of the brain demonstrated bilateral thalamic signal intensities. Extensive workup was again negative for bartonellosis, tularemia, rickettsioses, and rubeola. Normal studies included anti-NMDA, HSV, HHV-6, HHV-7, CMV, VZV, EBV, Mycoplasma, West Nile, Enterovirus, Typhus, HIV, NMO, MPO, PR-3, ANCA, ANA, HMPV, Adenovirus, Parainfluenza, Influenza. Parvovirus B19 CSF PCR was negative, but subsequent bloodwork had positive IgG and negative IgM, and positive serum PCR and bone marrow PCR at low levels with no pathologic evidence of acute parvovirus disease. Ferritin and soluble IL-2 were elevated, and natural killer cell count was low. A procedure was performed