Saxena, Reddy, Handa, and Singh: Ramsay hunt syndrome- expect the unexpected: A case report


Introduction

Infection with the Herpes zoster virus served as a potentially serious systemic disorder in the adults.1 Clinically, it is represented as a unilateral vesicular rash restricted to a single dermatome corresponding to the ganglion in which the dormant varicella zoster virus is reactivated. Skin lesions are associated with segmental neuralgia leads to pain and paresthesia.2 A special form of zoster infection of geniculate ganglion, with involvement of external ear, oral mucosa, peripheral facial nerve palsy accompanied by an erythematous vesicular rash on ear (zoster oticus) is termed as Ramsay Hunt syndrome.3

Case Report

A 56-year old male patient reported with pain in his left lower back tooth region since three to four days for which he took over the counter medication. He also complained of severe pain and ulcerations in mouth since one day. There was no relevant past medical and dental history. His vital parameters were within the normal range except 100°F body temperature. Extra-oral examination revealed pale to yellow-colored vesicles on left side of face distributed over lower lip, mandibular parasymphyseal region, cheek, pre-auricular region, post-auricular region, temporal region, external auditory meatus and tragus region (Figure 1, Figure 2).

Intra-oral examination revealed multiple ulcerations with encrustations on the left half of the lower labial mucosa, buccal mucosa, lower labial vestibule, buccal vestibule, left side of the tongue, floor of mouth, and soft palate with size ranging approximately from 0.1x0.1 cm to 1x1 cm (Figure 3, Figure 4, Figure 5). On hard tissue examination, severely attrited 34 with pulp exposure, root stumps of 13, disto-occlusal caries in 14, deep pocket in relation to 35 and grade I mobility in several teeth was present. 34 was tender on percussion. A provisional diagnosis of acute apical periodontitis with respect to 34 and other diagnosis of herpes zoster involving maxillary and mandibular branch of trigeminal nerve was made. Erythema multiforme, herpetic gingivostomatitis, erosive lichen planus, pemphigus and mucous membrane pemphigoid were considered as differential diagnosis.

The patient was prescribed with tab. valacyclovir (1000mg- three times daily), acyclovir cream (for topical application, four-five times daily), ibuprofen (400mg- two times daily), oint. Mucopain and warm saline gargles.

After five days, patient started complaining of drooling saliva and inability to close his left eye completely and numbness in the left side of face. Physical examination revealed that extra-oral lesions were partially dried up and intra-oral lesions reduced in size and extent. There was loss of wrinkles in the left side of forehead, drooping of the left eyelid and drooling saliva due to drooped left angle of mouth (Figure 6). Left pinna and external auditory meatus were swollen. A sharp shooting, lancinating pain was present arising from the left ear and radiating downwards along the course and distribution of the mandibular branch of trigeminal nerve, visual analog scale score was seven, indicative of neuralgic pain involving trigeminal nerve and facial palsy which is a feature of Ramsay Hunt syndrome. The patient was prescribed with prednisolone (30mg twice a day), Gabapentin (300mg -HS) and Carbamazepine (100mg- once a day) along with physiotherapy. After eight days, extra-oral and intra-oral lesions were healed. VAS score was five. Patient was advised to stop gabapentin. After 15 days, VAS score was four and symptoms of facial paralysis also reduced (Figure 7). Patient was advised to continue with prescribed medications along with tapered dose of prednisolone and to continue physiotherapy. After 20 days, patient was able to close his eyes completely and wrinkles were also reappeared over the left side of forehead. VAS score was three. Patient was advised to continue with carbamazepine-100mg. After 25 days, there was improvement in the symptoms of facial paralysis and pain with VAS score two. The dose of prednisolone was tapered and stopped. For next three visits with duration of seven days each, there was improvement in symptoms of facial paralysis but no relief in pain. The VAS score continued as two. A combination of gabapentin (300mg- HS for seven days), carbamazepine (100mg once a day for seven days) and neurobion forte (once a day for seven days) was prescribed. Based on the early diagnosis and combination therapy, high impact on better quality of life was seen.

Figure 1

Vesicles are present in the left side of face in the lower lip and cheek

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Figure 2

Vesicles are present in the left side post-auricular region

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Figure 3

Multiple ulceration present intraorally in the left side of lower labial mucosa

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Figure 4

Multiple ulceration present intraorally in the left side of tongue (dorsal and ventral surface)

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Figure 5

Multiple ulceration present intraorally in the left side of hard and soft palate

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Figure 6

Signs of facial palsy in the first recall visit

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Figure 7

Signs of facial palsy in the third recall visit

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Discussion

Herpes zoster is an acute infection with dermatomal eruption pattern. Reactivation of the virus can occur due to various triggering factors like advanced age, physical and psychological trauma, immunomodulatory medications etc.1 In our patient, maxillary and mandibular division were involved with the vesicular eruptions along the distribution of these three branches with facial palsy, vesicles of external ear, and loss of taste sensations in anterior two-third of the tongue, which are considered as key factors for the diagnosis of Ramsay Hunt syndrome. The early diagnosis and initiation of management is indicated in herpes zoster infection to alleviate symptoms and to prevent secondary infection. Management includes anti-virals which includes the acyclovir (800mg),2, 3, 4 valacyclovir (1000mg)1 and famcyclovir (500mg).5 But because of poor bioavailability,6 valacyclovir (1000mg three times a day) and famcyclovir (500mg three times a day) is the drug of choice for oral administration. Post-herpetic neuralgia can be managed with the use of anticonvulsants such as gabapentin, phenytoin, carbamazepine, and tricyclic antidepressants.5, 4 In the present case, management was done using antiviral drug along with anti-convulsant carbamazepine with a dose of 100mg per day and gabapentin (300mg per day). Later, when the patient developed signs and symptoms of facial palsy, corticosteroid was administered. Although corticosteroids are contraindicated in viral infections but they provide beneficial results in resolving facial palsy.

Conclusion

Ramsay Hunt syndrome is considered as an emergency condition and requires early diagnosis followed by management for a good prognosis and less long term adverse effects. Despite of diagnostic challenge, unilateral pattern of facial involvement and vesicular eruption helps in early diagnosis and differentiate the syndrome with diseases that mimicks other severe neurological diseases. The treatment should be initiated within 72 hours of the disease onset to prevent further morbidity. Antiviral therapy along with steroids should be initiated to prevent the disease progression, which lead to permanent neuronal damage.

Source of Funding

None.

Conflict of Interest

None.

References

1 

JK Brooks AM Rostami DC Mccorkle SI Benesh Trigeminal herpes zoster and Ramsay Hunt syndrome in an elderly adult: Presentation with prodromal toothacheGerodontology2018352768

2 

G Singh V Subhalakshmi S Balasubramanian M Patidar KK Ealla Ramsay hunt syndrome: A diagnostic challenge for general dental practitionersContemp Clin Dent201783379

3 

K Kayayurt O Yavasi O Bilir G Ersunan B Giakoup A Case of Ramsay Hunt Syndrome with Atypical PresentationTurk J Emerg Med20141431425

4 

U Malik M K Sunil C Gupta M Kumari Trigeminal Herpes Zoster: Early Recognition and Treatment -A Case ReportJ Indian Acad Oral Med Radiol2017293503

5 

SS Raj P Verma P Mahajan A Puri Herpes zoster infection of the face: A case report with review of literatureJ Indian Acad Oral Med Radiol20172915961

6 

H Handa BG Dara GS Naidu A Deshpande Zoster and its lurking shadowJ Indian Acad Oral Med Radiol201628337



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Article History

Received : 25-06-2021

Accepted : 26-06-2021

Available online : 09-10-2021


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https://doi.org/10.18231/j.jooo.2021.052


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