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Scenario 2: An atypical presentation

Sarah’s story

An illustration of a patient file, with the patients’ name (Sarah) and age (47) written on it

Sarah* is 47 years old and presented to accident and emergency (A&E) with severe, sudden-onset pain around her right ear. Read more about her history and experience with shingles.

*Not patient's real name. This is a hypothetical case for educational purposes only and does not replace clinical judgement.

Patient history

Sarah has no significant medical history and no ongoing medication usage.

Clinical presentation

Sarah presented to her local A&E department with severe, sudden-onset pain localised around her right ear. Sarah described her symptoms:

An illustration of a woman talking. She is saying “I have pain around my right ear that feels like it’s burning. I also feel dizzy and I’ve been having trouble staying upright and balancing, and my mouth feels like it’s difficult to move”

During the physical examination, you observe:

  • Redness and swelling at the right ear, extending into the ear canal
  • A single blister with a red base on the outside of the ear
  • Facial palsy on one side, drooping mouth corner, incomplete eyelid closure and reduced forehead wrinkles
  • Slightly unsteady gait

Which aspects of Sarah’s presentation might raise your clinical suspicion that her symptoms could be caused by shingles?

  • Burning nature of the pain

    The description of Sarah’s pain as being around just one ear and ‘burning’ in nature raises the suspicion of shingles.1

  • Vertigo

    Sarah describes feelings of vertigo, which could be a complication of shingles lesions in the ear or on the tympanic membrane.1

  • Facial palsy

    Sarah says that her mouth has felt difficult to move. In cases of shingles where the facial or auditory nerves are affected, this can present with ipsilateral facial palsy.1

Diagnosis

Following a comprehensive and multidisciplinary assessment, including polymerase chain reaction (PCR) testing of a skin swab obtained from the blister in Sarah’s ear, Sarah was diagnosed with herpes zoster oticus, also known as Ramsay Hunt syndrome.2,3 Treatment commenced immediately.

Ramsay Hunt Syndrome

Ramsay Hunt Syndrome occurs due to VZV infection spreading from the facial nerve to the vestibulocochlear nerve.2,3 Although clinical presentation varies, it typically presents with pain in the ear canal, peripheral facial weakness and auricular vesicular rash, but sometimes there is no rash at all.1,3,4 Other possible symptoms include vertigo (rotational dizziness), hearing loss, sensitivity to sound, tinnitus, dysgeusia, loss of taste and osteonecrosis.1,3,4

Involving the wider team

Patients like Sarah with herpes zoster oticus may develop a range of other complications – including hearing loss, sensitivity to sound, tinnitus, dysgeusia, loss of taste and osteonecrosis – as well as the ear pain, vertigo and facial palsy that Sarah was experiencing.1,3,4 What referrals might you consider for patients with this condition?

  • Otorhinolaryngology (ear, nose and throat [ENT])

    Otorhinolaryngologists may support in cases of shingles oticus, especially if facial or auditory nerves are involved.1,5

  • Neurology

    Neurologists can support in the case of acute focal neurological dysfunction or other neurological signs and symptoms.1,6

  • Pain management

    Pain specialists may support, for example, patients with persistent or inadequately controlled pain.5-7

Key learnings: Sarah’s case

An icon of the central nervous system

Herpes zoster oticus occurs when shingles spreads from the facial nerve to the ear, which can result in a variable clinical presentation.2

An icon of a woman

Sarah presented without the most typical skin lesions associated with shingles, making diagnosis more difficult. It is therefore important to be aware of other possible presentations of shingles and its complications.

An icon of a team of doctors

Although many patients with shingles will likely be managed in primary care by their general practitioners, a variety of medical specialists – for example ophthalmologists, pain specialists, otorhinolaryngologists, clinical immunologists, dermatologists and obstetricians – may need to be involved in patient care6,7

References

  1. Werner RN et al. European consensus-based (S2k) guideline on the management of herpes zoster - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV), Part 1: Diagnosis. J Eur Acad Dermatol Venereol 2017;31:9-19.
  2. Nair PA and Patel BC. Herpes zoster. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2024. https://www.ncbi.nlm.nih.gov/pubmed/28722854 (accessed February 2024).
  3. Harpaz R et al. Prevention of herpes zoster: Recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Recomm Rep 2008;57:1-30: quiz CE2-4.
  4. Crouch AE et al. Ramsay Hunt Syndrome. In: StatPearls. Treasure Island (FL): StatPearls Publishing. Available from https://www.ncbi.nlm.nih.gov/books/NBK557409/; 2024.
  5. Werner RN et al. European consensus-based (S2k) guideline on the management of herpes zoster - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV), Part 2: Treatment. J Eur Acad Dermatol Venereol 2017;31:20-29
  6. Johnson RW et al. Herpes zoster epidemiology, management, and disease and economic burden in Europe: A multidisciplinary perspective. Ther Adv Vaccines 2015;3:109-120.
  7. National Institute for Health and Care Excellence. Clinical knowledge summary: Shingles. https://cks.nice.org.uk/topics/shingles/ (accessed February 2024).
  8. Centers for Disease Control and Prevention. Zoster. https://www.cdc.gov/vaccines/pubs/pinkbook/herpes-zoster.html (accessed February 2024).

Adverse events should be reported. Reporting forms and information can be found at https://yellowcard.mhra.gov.uk/. Adverse events should also be reported to GlaxoSmithKline on 0800 221 441.

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July 2024 | NP-GB-HZU-WCNT-240021 (V1.0)