Abstract
Ramsay Hunt syndrome (RHS) is a complication of latent varicella-zoster virus infection, and is considered as a form of herpes zoster (HZ) occurring in the otic region. Although skin lesions of HZ usually appear along only one or two dermatomes, it is occasionally disseminated in patients with decreased immunity. Here we report a rare case of RHS developing disseminated herpes zoster in an immunocompetent young adult. A 16-year-old healthy man complained of left-sided facial weakness accompanied with otalgia, vesicles on the left ear, hearing loss, and vertigo. Although famciclovir and prednisolone was administered, his symptoms progressed and was hospitalized. Rashes all over the body developed, and dermatologist diagnosed a disseminated herpes zoster infection. Quarantine was commanded to prevent airborne transmission, and the administration of antiviral drug and steroid was continued. The patient recovered without sequelae. The important learning point of this case is discrimination from drug eruption for clinical decision-making.
Introduction
Ramsay Hunt syndrome (RHS) is a complication of latent varicella-zoster virus (VZV) infection, and is considered as a form of herpes zoster occurring in the otic region. It is usually identified clinically as a triad of facial paralysis, otalgia and herpetiform vesicles on the ear or in the mouth, frequently accompanied by vestibulocochlear symptoms, such as tinnitus, sensorineural hearing loss, and vertigo [Citation1]. Herpes zoster is caused by reactivation of VZV which may be dormant in sensory ganglia of the dorsal root ganglia after a previous varicella infection. Although grouped vesiculobullous skin lesions usually appear along only one or two dermatomes, it is occasionally disseminated hematogenously in patients with decreased immunity [Citation2]. We report a rare case of Ramsay Hunt syndrome developing disseminated herpes zoster in an immunocompetent young adult, in whom discrimination from drug eruption was important for clinical decision-making.
Case report
A 16-year-old male, without remarkable medical history, visited our hospital complaining of left-sided facial weakness and hearing loss for 1 day. Three days before, the patient experienced left otalgia and spinning vertigo. Tinnitus and headache were absent. There was no remarkable sick contact. It was one year before the onset of COVID-19 pandemic. He had suffered chickenpox during childhood. Physical examination revealed multiple vesicles on the left pinna. Facial palsy with House-Brackmann Grade III [Citation3] was noted on the left side. Spontaneous nystagmus was not noted. Audiometry showed slight high-tone hearing loss in the left ear (Figure ). The routine blood test was normal including inflammatory markers (WBC 7,900/microL and CRP 0.02 mg/dL). With the diagnosis of Ramsay Hunt syndrome, famciclovir (1500 mg/day) and prednisolone (50 mg/day) was prescribed.
Figure 1. Audiogram on the first visit.
However, the symptoms gradually worsened. He became unable to walk due to vertigo with emesis, rashes developed all over the body, and so was hospitalized on the 7th day of onset. Facial palsy progressed to House-Brackmann Grade IV and rightward beating nystagmus was observed. Serological tests revealed elevated anti-VZV IgG (18.5: normal range 0–1.9) but normal anti-VZV IgM (0.32: normal range: 0–0.79), compatible with reactivation of VZV. Several erythematous papules with vesicles were observed on his chest, abdomen, back, both arms and legs, along with those on the left earlobe. Intraoral vesicles were not noted. We suspected drug eruption and consulted an experienced dermatologist, who diagnosed a disseminated herpes zoster infection and commanded quarantine to prevent airborne transmission. Intravenous prednisolone (40 mg/day) was administered and oral famciclovir (1500 mg/day) was continued. The patient was discharged after crusting of the rash. Photographs of the skin eruptions with crusting are shown in Figure . House-Brackmann Grades were II on the 26th day and I on the 52nd day of onset. The whole clinical course is shown in Figure .
Figure 2. Skin rashes with crusting (ear lobe, arm, chest, and back).
Figure 3. Course of symptoms and drug administration. PSL: prednisolone.
Discussion
Herpes zoster is a consequence of reactivation of latent VZV from the dorsal root ganglia. It is characterized by unilateral vesicular eruptions within one or two dermatomes. Disseminated herpes zoster has been defined as more than 20 vesicles outside the area of the primary and adjacent dermatomes [Citation4], and is very rare with immunocompetent subjects, in contrast to a higher incidence (10–40%) in patients with decreased immunity [Citation5]. The dissemination occurs 4 to 11 days after the onset of localized lesions. [Citation6]
Patients with disseminated herpes zoster should take antiviral medicine and they must be quarantined to prevent transmission of airborne infection until all lesions have crusted [Citation7]. The Center for Disease Control and Prevention(CDC) has recommended that all patients with varicella or disseminated zoster be placed in rooms meeting requirements suitable to house patients with tuberculosis (private room negative pressure of room with respect, 6 air exchanges per hour of more, and air exhausted directly to the outside) [Citation8].
Disseminated herpes zoster with Ramsay Hunt syndrome is also rare. Of the 6 reported cases including ours (Table ) [Citation9–13], age ranged from 16 to 75, and the present patient is one of the youngest. Cases listed on the Table were limited to those with sufficient description of otological findings in whom dissemination followed the onset of Ramsay Hunt Syndrome. All 6 cases were male and 3 non immunocompromised patients were included. Cutaneous dissemination occurred in 4–11 days after the initial dermatomal skin rash. Dissemination can occur both before and after the involvement of facial nerve. Facial palsy recovered in all 3 immunocompetent patients.
Table 1. Reported cases with Ramsay Hunt syndrome developing disseminated herpes zoster.
Drug eruption has various types, e.g. morbilliform, urticarial, etc., which are non-specific for drug classification. It sometimes needs differentiation from herpetic vesicles of VZV by the experienced dermatologist. The incidence of skin rash as an adverse effect in the phase III study of famciclovir in our country was 0.9% [Citation14]. Therefore, drug eruption is not common, either. However, disseminated herpes zoster with Ramsay Hunt syndrome among immunocompetent subjects must be even rarer, since only 3 definite cases have been reported. The differential diagnosis of the rash is important for clinical decision-making, since the antiviral drug should be continued with quarantine in case of exacerbation of VZV infection, but be withdrawn in case of a drug side effect. Although direct immunofluorescence assay for VZV antigen or polymerase-chain-reaction (PCR) assay VZV DNA can make definite diagnosis, these are not universally available and may take time according to the facilities. In the present case, diagnosis of disseminated herpes zoster was made promptly by the dermatologist. Its course of simultaneous appearance with exacerbation of symptoms and disappearance after crusting with continued administration of the antiviral drug was typical with disseminated herpes.
Conclusion
A rare case of Ramsay Hunt syndrome with disseminated herpes zoster in an immunocompetent young adult was reported. Quarantine was commanded to prevent airborne transmission with continuation of antiviral drug. Discrimination from drug eruption was important for clinical decision-making.
Informed consent
Written informed consent was obtained from the patient for the publication of any potentially identifiable images or data included in this article.
Ethical approval
The presentation of this case was approved by the institutional review board of our university (19-074-4).
Disclosure statement
No potential conflict of interest was reported by the authors.
References
- Sweeney CJ, Gilden DH. Ramsay hunt syndrome. J Neurol Neurosurg Psychiatry. 2001;71(2):149–154.
- Downe AW. Chickenpox and zoster. Br Med Bull. 1959;15:197–200.
- House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg. 1985;93:46–147.
- Whitley RJ, Gnann JW, Hinthorn D, et al. Disseminated herpes zoster in the immunocompromised host: a comparative trial of acyclovir and vidarabine. The NIAID collaborative antiviral study group. J Infect Dis. 1992;165(3):450–455.
- Gupta S, Jain A, Gardiner C, et al. A rare case of disseminated cutaneous zoster in an immunocompetent patient. BMC Fam Pract. 2005;6:50.
- Mazur MH, Dolin R. Herpes zoster at the NIL: a 20 year experience. Am J Med. 1978;65(5):738–744.
- Cohen JI, Zoster H. Clinical practice: herpes zoster. N Engl J Med. 2013;369(3):255–263.
- Weber DJ, Rutala WA, Hamilton H. Prevention and control of varicella-zoster infections in healthcare facilities. Infect Control Hosp Epidemiol. 1996;17(10):694–705.
- Nigam P, Dayal SG, Dubey AL. Disseminated zoster. J Dermatol. 1980;7(6):443–447.
- Yoon KJ, Kim SH, Lee EH, et al. Disseminated herpes zoster in an immunocompetent elderly patient. Korean J Pain. 2013 April;26(2):195–198.
- Lee EJ, Yoon YJ. A case of generalized varicelliform eruption after herpes zoster oticus. Otol Neurotol. 2014;35(1):e57–59–e59.
- Chen I, Fohtung RB, Oughli HA, et al. Concurrent ramsay hunt syndrome and disseminated herpes zoster in a patient with relapsed chronic lymphocytic leukemia. IDCases. 2016;6:79–82.
- Rissardo JP, Caprara ANLF. Herpes zoster oticus, opthalmicus, and cutaneous disseminated: case report and literature review. Neuro-ophthalmology. 2019;43(6):407–410.
- Honda M, Tamaki K, Igarashi A. Phase III study of famciclovir tablet in patients with herpes zoster – double-blind comparative study with aciclovir. Rinsho Iyaku. 2008;24(9):825–848.