SAEM Clinical Images Series: A Rash with Cranial Nerve Deficits
A 48-year-old male with no significant past medical history presented to the Emergency Department with a left-sided facial rash and associated burning left eye pain that started four days prior. He was seen at an ophthalmology clinic when his symptoms started and given oral valacyclovir which he took for three days without improvement. He also endorsed left-sided facial weakness and diplopia for the last eight days. He denied fevers, chills, nausea, vomiting, ear pain, tinnitus, hearing changes, blurry vision, photophobia, history of malignancy or HIV, history of stroke. He reported remote use of tobacco nine months prior, cocaine use that stopped three weeks prior, and alcohol use only on weekends.
Vitals: Temp 36.8°C; BP 148/85; HR 80; RR 18; O2 Sat 96% on room air
General: Alert, no acute distress.
Skin: Healed vesicular rash along V2 distribution of trigeminal nerve.
Head: Normocephalic, atraumatic.
Eye: Visual acuity – right 20/40, left 20/70 without correction (at baseline per patient). Left eye viewed with fluorescein showing dendritic lesions. EOM: right intact, impaired abduction of left eye. Unable to close the left lid. Pupils: R pupil 3mm, briskly reactive to light, L pupil 3mm not briskly reactive
Ears: Without vesicular lesions bilaterally
Cardiovascular: Normal peripheral perfusion.
Respiratory: Respirations are non-labored.
Neurological: Alert and oriented to person, place, time, and situation. Cranial nerves: CN II grossly intact, CN III: left pupil reactive to light but sluggish, CN V: facial sensation to light touch intact, CN VI: impaired abduction of left eye, CN VII: left facial droop with left forehead involved, CN VIII – XII intact. 5/5 motor strength to bilateral upper and lower extremities, no sensory deficits, has a steady gait.
CBC, BMP, and ESR all within normal limits.
The patient has a left-sided painful vesicular rash in the V2 distribution of the trigeminal nerve and dendrites on fluorescein-stained exam of the left eye, concerning for herpes zoster ophthalmicus. Hutchinson sign (involvement of the tip or side of the nose, as seen in the images) indicates involvement of the nasociliary branch of the trigeminal nerve, and patients with this finding have an increased risk of ocular involvement [1]. Although this patient did not have auditory canal involvement, Ramsay Hunt Syndrome is also important to consider. The image also shows impaired abduction of the left eye, concerning for a CN VI palsy, and the physical exam showed sluggish left pupil reactivity to light, concerning for a CN III palsy. Given the patient reported diplopia and had multiple cranial nerve deficits, cavernous sinus syndrome was also a differential diagnosis.
The recommended treatment for herpes zoster ophthalmicus is oral Valacyclovir, however, if there is any concern for disseminated zoster (3 or more dermatomes involved, CNS involvement, or other extradermal complications), patients should be treated with IV Acyclovir 10 mg/kg based on ideal body weight every 6 hours [1]. Since this patient underwent a trial of Valacyclovir without improvement and there was concern for possible CNS involvement with multiple cranial nerve deficits on exam, the patient was started on IV Acyclovir. Consultation with ophthalmology is also recommended for management of zoster ophthalmicus. The presence of diplopia, CN III and CN VI palsies was also concerning for possible cavernous sinus syndrome, which can be caused by a broad range of infectious, inflammatory, neoplastic, and vascular pathologies. It can have varying presentations based on the affected neurovascular structures however the constellation of symptoms includes diplopia, ophthalmoplegia, Horner syndrome, facial sensory loss, and CN III, VI, and VI deficits. If it is suspected, MRI with and without contrast is the preferred imaging modality to determine the location and extent of disease [2]. Neurology consultation is also helpful in co-management.
Take-Home Points
- In a patient with a facial vesicular rash, it is important to perform a full cranial nerve exam to evaluate for deficits that may indicate CNS involvement, inspect the anterior chamber to evaluate for zoster ophthalmicus, and examine the ears to evaluate for Ramsay Hunt Syndrome.
- Start antivirals early if zoster ophthalmicus is suspected since this disease process can be vision-threatening.
- Anderson Erik, Do-Nguyen Amy. Varicella-Zoster Virus (VZV). In: Mattu A and Swadron S, ed. CorePendium. Burbank, CA: CorePendium, LLC. https://www.emrap.org/ corependium/chapter/recV6PonFTQbz5R9c/Varicella-Zoster-Virus-VZV#h.q140vny9bkbo. Updated August 16, 2023. Accessed January 11, 2024.
- Munawar K, Nayak G, Fatterpekar GM, et al. Cavernous sinus lesions. Clinical Imaging. 2020;68:71-89. doi:https://doi.org/10.1016/ j.clinimag.2020.06.029