Erythroderma and Eosinophilia with Multi-System Involvement, a Case of Vress

Introduction: Resembling Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), Virus Reactivation with Eosinophilia and Systemic Symptoms (VRESS) occurs triggered by members of Herpesviridae family viruses. Case Description: A 12-year-old male was hospitalized with a 5-day history of fever, pruritic erythematous rash on the trunk and extremities, and facial edema. The patient was exposed to a cousin diagnosed with Multisystem Inflammatory Syndrome in Children (MIS-C) 10 days prior. No history of traveling, unusual food intake, URI, drug ingestion during the prior 3 months. Patient was treated with clindamycin x 10 days for possible Staphylococcal Scalded Skin Syndrome. Patient was noted to have eosinophilia of >2000 that persisted through the hospital course and thereafter, transaminitis with direct bilirubinemia, transient microscopic hematuria, elevated CRP and C3, normal EKG and chest radiograph, normal T/B/NKC counts, normal levels of immunoglobulins and elevated IgE (2163>1579IU/mL). IgG and IgM were negative for: CMV, adenovirus, strongyloidces, HAV, HBV, and HIV. IgG positive to: EBV, HHV-6, mycoplasma, HHV-6, HSV1, and parvovirus. Negative cultures for MRSA and group A Streptococcus. Negative PCRs for COVID -19. At 8 week follow up, skin erythema evolved into desquamation in the fingers and eosinophilia persisted. Discussion(s): VRESS should be on the differential for patients presenting with a DRESS-like symptoms in the absence of drug exposures. VRESS is often triggered by members of the Herpesviridae family of viruses including EBV and HHV-6, to both of which this patient was exposed to in the past. While diagnosis is one of exclusion, early identification can guide appropriate management. Copyright © 2022

A Crick in theNeck: Lemierre Syndrome in a 4-year Old Female Presenting as Fever and Torticollis

Introduction: Described by Dr. Andre Lemierre in a 1936 case series of 20 patients, Lemierre Syndrome (LS) is defined as a septic thrombophlebitis of the internal jugular vein (IJV). LS typically begins as an oropharyngeal infection that advances to the IJV through direct extension through the fascial planes of the neck, or indirect lymphatic or hematogenous spread from the peritonsillar vessels. We present a case of LS in a 4-year-old patient who presents much younger than the typical age range of affected individuals, and who recovered well without any longterm sequelae. Case Description: A 4-year-old ex-27 week female presented with a near 3 week history of intermittent fevers and progressive right-facing torticollis. She had multiple interactions with the health care system over her illness course, and was given diagnoses ranging from general viral syndrome to gingivostomatitis and acute otitis media. Around the 2 week mark, her caretaker described her as having developed a “crick” in her neck while consistently favoring a rightward tilt. On illness day 16, she presented to her pediatrician for routine visit, and was noted to have fever, right tonsillar enlargement, and cervical lymphadenopathy, thereby prompting referral to the emergency department. Her physical exam on admission was additionally significant for a 30 degree rightward head rotation, a swollen and tender right sternocleidomastoid, and submandibular lymphadenopathy. She was resistant to active or passive neck rotation due to discomfort, but was able to traverse the midline with coaxing. Laboratory workup was notable for leukocytosis and thrombocytosis with elevated inflammatory markers, as well as mild transaminitis. Infectious serologic workup was negative for: SARS-CoV-2, Bartonella henselae, Bartonella quintana, EBV, and Mycoplasma. Blood culture showed no growth, but was drawn after antibiotics were given. A CT neck with contrast demonstrated intrinsic occlusion vs compression of the right IJV, and ultrasound and MRI confirmed IJV thrombophlebitis. Discussion: LS is typically associated with Fusobacterium necrophorum infection, a gram-negative anaerobe, with incidence estimated to be around 1 to 3.6 per million per year and mortality rate around 5 to 9%. Significant morbidity is often present, due to dissemination of septic thromboemboli, potentially affecting the CNS, bones/joints, and lungs. The typical age range for LS in pediatric patients clusters around adolescence, but infants as young as 6 months of age have been reported. As oropharyngeal infections most often precede LS, it is important to keep this rare but serious infection on any differential. Conclusion: This patient was diagnosed with Lemierre Syndrome. She was treated with an inpatient course of ampicillin/sulbactam before transitioning to oral amoxicillin/clavulanic acid to complete a total of 4 weeks of antibiotic therapy. All elevated laboratory markers normalized prior to hospital discharge, and the patient had complete resolution of symptoms at outpatient follow up.