Laryngeal sarcoidosis—A case report

doi:10.4236/crcm.2013.26100

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Vol.2, No.6, 375-376 (2013) Case Reports in Clinical Medicine

http://dx.doi.org/10.4236/crcm.2013.26100

Laryngeal sarcoidosis—A case report*

Hemanth Kowdley Subrahmanyam#, P. Gana, R. J. Lee

Department of ENT-Head & Neck Surgery, Kettering General Hospital, Kettering, UK; #Corresponding Author: [email protected]

Received 19 July 2013; revised 5 August 2013; accepted 12 August 2013

Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is

properly cited.

ABSTRACT

Laryngeal sarcoidosis while involvement of the

vocal cords in the disease process is excep-

tionally rare. Involvement of the larynx is fre-

quently limited to the supraglottis that is rich in

lympho epithelial tissue and usually presents

with difficulty in breathing of varying severity.

We present a rare case of isolated sarcoidosis

involving the vocal cord in a patient with the

previous history of cutaneous sarcoidosis.

Keywords: Laryngeal Sarcoidosis

1. INTRODUCTION

Sarcoidosis is a chronic granulomatous disease involv-

ing in multiple organs in the body. Common sites of in-

volvement include lungs, hilar & mediastinal lymph

nodes, liver, eyes, skin, bone and the nervous system.

Diagnosis is based on a correlation between the clinical,

radiological and histological findings [1]. Supraglottic

involvement is a very common finding in the larynx.

True vocal cord involvement is a rarity. We present an

interesting case report who presented to us with chronic

cough as the only primary laryngeal symptom.

2. CASE REPORT

A 36-year-old female Caucasian smoker was referred

by respiratory physicians with complaints of hoarseness

for 8 months duration. She had received multiple courses

of antibiotics for a presumed URTI with no effect on her

hoarseness. There was no odynophagia or dysphagia. She

had no loss of weight or appetite. No history of voice

abuse was recorded. She was diagnosed with cutaneous

sarcoidosis in 2002. Her co-morbidities included hay

fever and Type II diabetes. Flexible nasolaryngoscopy

revealed left vocal cord and ventricular band thickening.

Her ACE level was elevated and CXR revealed bilateral

hilar lymph node enlargement signifying an active period

of her systemic sarcoidosis. Microlaryngoscopy and bi-

opsy suggested a narrow glottic area. Biopsy of the left

vocal cord revealed features consistent with Sarcoidosis

showing florid granulomatous inflammation alongside

well formed epitheloid granulomas and occasional mul-

tinucleated giant cells (Figure 1). There was no evidence

of caseous necrosis or malignancy. A diagnosis of laryn-

geal sarcoidosis was made. Treatment with inhalational

and systemic corticosteroids significantly improved her

condition. She has symptomatically remained stable and

on regular follow up.

3. DISCUSSION

The first confirmed case of laryngeal sarcoidosis was

by Poe in 1940 according to the literature [3]. Sarcoido-

sis is an idiopathic phenomenom characterised by non

caseating granulomas. Prevalence does vary in the ethnic

groups and is found to be 50 per 100,000 in Scandina-

vian countries and African Americans. It is a multi sys-

tem disorder which typically affects population between

20 and 40 years of age. Laryngeal sarcoidosis prevalence

is 0.5% to 8.3% in patients with a past history of sarcoi-

dosis [1]. It affects the supraglottis typically with isolated

evidence affecting the glottis. Interestingly it is theorized

that sarcoidosis affects the reticuloendothelial system and

hence vocal fold involvement is relatively uncommon

due to lack of lymphoid tissue [1,4].

Common s ymp toms at pre senta tion ar e hoar senes s and

stridor. Less commonly dysphagia, globus and cough

could be presenting features. Disease does tend to fluctu-

ate between active and latent periods [2] and therefore

calls for prolonged per iod of follow up. In the supraglot-

tis, epiglottis is the most common site of involvement

though any part of larynx could be affected [4]. Hoarse-

ness could be due to direct vocal fold involvement or as a

result of peripheral neuropathy affecting the vocal folds

and can usually be confirmed by laryngeal EMG [1].

Diagnosis is essentially established by histology demon-

trating non caeseous granulomatous inflammation thus s

*Conflict of interest: Nil.

H. K. Subrahmanyam et al. / Case Reports in Clinical Medicine 2 (2 013) 375-376

376

Figure 1. Well formed epitheloid granulomas with florid granulomatous inflammation and oc-

casional multinucleated giant cells suggestive of sarcoidosis (arrow).

excluding other causes of granulomatous laryngitis [4].

The disease however, being multi-systemic in nature,

ancillary screening tests such as such chest X-ray, fbc,

serum calcium levels, liver and renal function are neces-

sary [1].

Treatment regimen includes systemic steroids, intrale-

sional steroids for well localised lesions, laser resection,

surgical excision, low dose radiation and tracheostomy.

Recent literature has suggested an improvement with use

of inhaled steroid and Clofazimine [4]. However treat-

ment modality does depend on the degree and sev erity of

sy mptoms.

4. CONCLUSION

Sarcoidosis is found to affect mainly the reticuloendo-

thelial system, thus in this case vocal cord involvement

makes it a rare and interesting presentation. As this case

and review of literature suggest that there is little evi-

dence to forecast the prognosis due to paucity of reported

cases and limited duration of follow-up. The role of com-

bined modalities of treatment of laryngeal sarcoid re-

quires further objective assessment. In view of the pro-

pensity to relapse, a close and long term follow-up is

essential.

REFERENCES

[1] Schwartzbauer, H.R. and Tami, H.A. (2003) Ear nose and

throat manifestations of sarcoidosis. Otolaryngologic Cli-

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doi:10.1016/S0030-6665(03)00030-6

[2] Dean, C.M., Sataloff, R.T., Hawkshaw, M. J. and Pribikin,

E. (2002) Laryngeal sarcoidosis. Journal of Voice, 16,

283-288. doi:10.1016/S0892-1997(02)00099-1

[3] Poe, D.L. (1940) Sarcoidosis of the larynx. Archives of

Otolaryngology, 32, 315-320.

doi:10.1001/archotol.1940.00660020318007

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med, U.R., Rashmikant, D. and Padmanabhan, K. (2005)

Persistent hoarseness of voice in a sarcoid patient: A clue

to laryngeal sarcoidosis; a potential cause of upper airway

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