Age: 23.0
Sex: F
Vaccine: HEPAB, TD, YF, HEPAB, TD, YF, HEP, HEPA, TD, YF
Symptoms: Rash pruritic, Skin ulcer, Somnolence, Speech disorder, Tenderness
Clinical narrative: Per the reporter, a 23 year old female subject with a previous diagnosis of systemic cutaneous lupus erythematous and partial C4 deficiency since age six, allergies to cephalosporins, penicillin and sulfa drugs, recurrent ear infections and removal of vocal cord nodule as a child, had received an administration of YF Vax vaccine, (lot #, route and site not reported), an administration of Diphtheria and tetanus toxoids (manufacturer, lot number, route and site not reported) and an administration of Hepatitis A & B vaccine (manufacturer, lot number, route and site not reported), on 1/27/2006 in preparation for travel to out of country. The patients treatment for past medical conditions have included naproxen and analgesic drugs. When she was 19 years old she had a flare up of symptoms that involved the joints and skin without any other systemic manifestations of the SLE. She had used methotrexate and prednisone 5mg/d in the past. Since Jan 2005 she had just been using hydroxychloroquine. She is a vegetarian, works with dogs and denies any drug abuse. on 1/2/2006, five days post vaccination, the patient developed a fever (up to 102.5 F) and three days later, she presented to the ER for treatment for her fever. Concomitant symptoms included, chills, nausea, vomiting, fatigue, myalgias, arthralgias (different from SLE), history of one episode of loose bowel movement, one day history of diffuse maculopapular generalized rash and just feeling bad. The patient had been taking Tylenol PRN for the fever, Plaquenil and Yasmin OCP. On the morning of presentation, her symptoms had improved, but she noticed pruritic rash on her trunk and extremities. She reported feeling itchy the night before the onset of the rash. She denied any sick contacts. her review of systems revealed a fever, intermittent tinnitus, blacks spots in the visual fields, slight tenderness to palpitation in the mid lower abdomen with no rebound or guarding, a blanching maculopapular rash on her trunk, extremities and face and petechiae on her upper palate. Lab work completed and the results revealed WBC 3.5 (N55, L27, E6, B1, M11), HgB 3, HCT 37, Platelets 173, NA 138, K+ 3.5, CL 104, Bicarb 26, Gluc 104, BUN 7, CR 0.7, AST 25, ALT 25, Albumin 3.3, U/A neg, Blood Cx x2 results ?, ANA 8 (nl <7.5 speckled, C3 129 (WNL, C4 <1 (nl 16-70), ESR 0, dsDNA neg, anti cardiolipin IgG and IgA = neg. IgM 21 (20/80 = low medium positive). The patient was treated with Benadryl and Tylenol for suspected allergic reaction. there was a plan to give her Prednisone 10mg x 1 but this was reportedly not given. She was kept overnight for observation. the next day, on 2/5/2006, she remained afebrile and her rash was noted to be 80% improved after the Benadryl. Her Maculopapular rash was predominantly on her chest and arms. She was seen by an allergist who felt the rash was likely to be viral exanthema. She was discharged from the hospital with Benadryl and was given permission to travel. Discharge lab results were WBC .34 (N49, E7, B1, L33, M10) PLT 164, INR 1.1, PTT 26.3. On 2/7/2006 the patient left on trip out of country. During the air flight she developed a headache that became increasingly severe in nature. On 2/11/2006 she consulted a physician at a hospital for her headache. During the medical consultation she developed a generalized tonic clonic seizure. She was admitted to the hospital. At that time she had no fever or rash but demonstrated global aphasia without motor deficits or other neurologic clinical abnormalities. The attending neurologist diagnosed her with post seizure aphasia. Her condition improved and she was able to answer the questions asked her. At the time the patient had no pain and was only mildly irritated and agitated. A CT of the head showed sulcus were not marked indicating cerebral edema, no hemorrhage and a suspected low density image which could indicate an old lesion (?CNS lupus, ? APS). Lab work up revealed the patients WBC to be 150 (polymorph nuclear predominance, PTN 40 mg, Gluc 49 mg and the CSF pleocytosis with 150 cells 77% PMN. No other abnormalities in the lab test were noted except leucocytosis with normal differential count. The patient was admitted to the intensive care unit and was treated with acyclovir initially, but then it was discontinued when her HSV PCR was returned negative, meronem, steroids, phenytoin and IV fluids. The patient reportedly recovered quickly from the seizure, and she remained alert with no focal deficiency signs. The next day, on 2/12/2006, the patients mental status was normal as was the rest of her clinical examination. Her chief complaint was a headache. An MRI showed no abnormalities (ADEM (acute disseminating encephalomyelitis) not probable) and her serum amylase, lipase and CPK were normal. The following day, the patient experienced agitation, mental confusion, poor speech with no motor deficits. She complained of a headache and abdominal pain. There was no sign of peritoneal irritation and her bowel sounds were present. lab results revealed amylase 84 U/l, lipase 81 U/L, LDH 436 u/l, all normal. Her Anti DNA ds, ANA were negative, C3 was normal (serum was negative, lupus was still a concern but not probable), and her C4 was close to the lower limit of normal range. On 2/14/2006, the patient was complaining of muscle pain, headache and abdominal pain. She continued to present with mental confusion but had no fever. Her amylase was 1231 U/L, Lipase 8633 U/l, LDH 1006 U/L, CPK 6437, Myoglobin 707. An anterior posterior CT scan without contrast showed a pancreas volume increase with discrete tissue infiltration and free fluid in the abdominal cavity that could not be attributed to overload of fluid administration. Her IgG and IgM were negative for herpes and the acyclovir was stopped. Anti SSA, SS-B, and S-M were negative and so lupus was not considered. The physicians impression was that the patient had pancreatitis (? vaccine reaction, ? viral, ? phenytoin). The Rhabdomyolysis was too far from the seizure so it was considered probable myositis. The physician was now considering yellow fever reaction or viral infection as the only hypothesis discussed with the family. He was awaiting the serologic and molecular biologic Studies. Treatment provided was supportive to keep the urinary volume above 200ml/h and no food by mouth. She was transferred to the ICU of another hospital. On 2/15/2006, the patient was still complaining of muscle pain, headache and abdominal pain. She continued to present with mental confusion alternated with somnolence plus discrete central facial and brachial paresis but no fever. Her amylase was 200, lipase 1000, CPK 20,000 and stable. Her liver enzymes ALT and AST remained elevated and the mild myoglobinuria had resolved. Renal function remained normal. A brain MRI revealed no abnormalities except a discrete contrast enhancement in the meninges, but no e/o increased ICP ore focal lesions. Repeat DS DNA and C3 were WNL and C4 remained low. C reactive protein peaked at 10 (nl < 1.0), but had decreased to 7 (2/16). She had no skin lesions suggestive for SLE. WBC were elevated with normal differential and platelets and coagulation remained normal. There was no proteinuria. Serology for ESV, CMV, coxsackie, hepatitis B, herpes zoster adeno enterovirus were negative. The physicians impression was that the pot was worsening in terms of systemic evolvement. On 2/16/2006, the patient seemed much better without mental confusion and focal neurologic manifestations. All of the lab tests improved. She was administered fresh frozen plasma and had a minor reaction to the plasma so it was stopped. On 2/17/2006 the patient was doing pretty good. She was still complaining of a headache and muscle pain. She was being tested with some food by mouth carefully. On 2/21/2006 the patient was asymptomatic, hungry and without deep venous access, but she does have some signs and symptoms on her MRI of encephalitis. This is being investigates as a possible etiological agent.