A 71-year-old female transferred to The Johns Hopkins Hospital with rapid onset of fever and edema involving the face, upper extremities and chest, associated with hypotension.

The patient is a 71 year old woman with no significant past medical history who was in Baltimore visiting her son during April, 2002. Approximately 10 days prior to admission she traveled by train from Florida to visit with family in Baltimore. A few days after she arrived, she began to complain of nasal burning and a sore throat. Her sister-in-law gave her lidocaine topical gel to treat the burning. Her symptoms progressed over the next few days to include facial swelling involving the forehead and eyelids. Five days prior to admission, the patient was seen by family's physician who felt the patient was having an allergic reaction to lidocaine and she was treated with an intramuscular injection of steroids into the left arm. She also began a course of oral Prednisone 20 mg/day. It is not known if she had fevers, chills, or night sweats.

After the injection, the patient complained of worsening left arm pain and a diffuse erythematous truncal rash with swelling involving her left arm and upper chest. The following day, the patient developed systemic symptoms that included nausea and explosive diarrhea. The next morning, she was found somnolent but arousable in the bathroom. Her left upper extremity was profoundly swollen and she was unable to use her left hand. She was brought by ambulance to an outside hospital.

In the Emergency Department she had a temperature of 103° F and a systolic blood pressure of 80 mmHg. Dopamine, vancomycin, cephtriaxone, clindamycin and doxycycline were started. She was transferred by ambulance to The Johns Hopkins Hospital Medical Intensive Care Unit for further treatment.

No history of diabetes, myocardial infarction, stroke, vasculitis, or chronic infections. Her physician has told her she has borderline hypertension that is controlled with an active lifestyle and prudent diet.

None

Unremarkable

Retired schoolteacher living in South Florida. No recent history of tick bites, foreign travel, or gardening. Her only pet is a small dog. She does not smoke cigarettes and does not use illicit drugs. She drinks 3-5 glasses of sherry/week. Her hobbies include mah jong, tai chi, and water aerobics. She is widowed. She has unprotected sexual activity with a boyfriend.

Multivitamins

Sulfa drugs cause a rash

Ill, uncomfortable appearing female. Alert to person, place and time. Temperature is 38.3°. BP=80 mmHg by Doppler on dopamine and fluids. HR=110- 190 irreg/irreg. RR=40/min; 100% on 100% NRB
Head and Neck: Erythematous rash on forehead and eyelids
Mouth: Mild erythema of the pharynx and throat. No tonsillar exudates.
Cardiovascular: Tachycardic and irregular/irregular
Chest: Slight rhonchi. No wheezes, egophany, or crackles.
Abdomen: Soft, nontender without masses. Bowel sounds are present.
Extremities:
Left arm: swollen, blistering, desquamative rash. Bullae present, some blood filled and some with clear fluid. Left lower third of forearm through to fingertips are cold, cyanotic. No crepitus. Absent sensation to pain and paralysis from PIP joints to all digits of left hand. No radial pulse by Doppler. Brachial pulse is present by Doppler.
Right arm: cold distally from forearm, hand is cyanotic. Large erythematous area on dorsal aspect of right hand. Intact sensation and movement. Radial and brachial pulses identified by Doppler.
Bilateral lower extremites: Bilateral feet cold, pale with intact sensation and movement. Strong femoral pulses bilaterally. Popliteal pulses present by Doppler bilaterally.
Left foot: Pale, cold from ankle. Movement and sensation intact. Dorsalis pedis and posterior tibial pulses present by Doppler.
Right foot: Pale, cold from ankle. Movement and sensation are intact. Dorsalis pedis and posterior tibial pulses are absent by Doppler.

On day of admission: WBC=12,340, HGB=13.5, HCT=38.6, PLTs=120,000. Na+=136, K+=2.7, CL=110, CO2=37, Cr=1.3, BUN=37, Glucose=110, Ca++=5.4, Phos=2.4, Mg++=1.0, Lactate=4.8.
T Prot=2.4, Alb=1.2, T Bili=0.3, ALT=78, AST=168
PT/INR=15.2/1.3 PTT=45.7, d dimer=4.01, fibrinogen=401
CK=6861, MB fract=104, Troponin=0.46
Antistreptolysin O Test=43

Chest X-ray: Lung fields clear.

Atrial fibrillation

On admission, she was febrile, oxygenating poorly, and she had significant left arm edema with blistering and desquamation of the skin as well as cold, cyanotic fingertips and absent radial pulse. Her right arm and both legs were cold but pulses were identified. She was brought to the operating room by Plastic Surgery for emergent left upper extremity fasciotomy and debridement with restoration of her left radial pulse. Nine hours later her left radial pulse was lost and she was taken back to the operating room by Vascular Surgery for left brachial artery thrombectomy.

Postoperatively, the patient had worsening blood pressure requiring 26 liters of fluid over the 24 hour period. In addition, her abdomen progressively distended raising the possibility of abdominal compartment syndrome. She was taken back to the operating room and underwent temporary vacuum pack closure of the abdomen and bilateral lower extremity four compartment fasciotomies. The day following this surgery, the patient was found to have elevated cardiac enzymes consistent with acute myocardial infarction. She remained in critical condition over the next few days. Left upper extremity and right upper extremity ischemia progressively worsened and she was subsequently brought back to the operating room for partial amputation of the left upper extremity, debridement of the right upper extremity and washout of her abdomen. With the slow withdrawal of pain and sedative medications, it became apparent that the patient had developed a profound coma. Axial noncontrast head CT images (Images 1-4) showed multifocal cortically-based regions of hypodensity affecting the left occipitotemporal lobe, left medial occipital lobe, left parietal lobe, right parietal lobe, and in the right parietooccipital junction. Some regions had a wedge-shaped configuration. Mass effect was seen with effacement of sulci of the left cerebral hemisphere and compression on the left lateral ventricle.

Given the poor prognosis, a decision was made to make the patient DNR and the patient expired shortly thereafter.

What is the most likely cause of the patient's acute illness and subsequent hospital course?
What additional diagnostic tests would have been helpful?
Should any additional therapy have been considered early in her course?