Indian J Otolaryngol Head Neck Surg
(November 2019) 71(Suppl 2):S1027–S1029; DOI 10.1007/s12070-016-0964-6
CLINICAL REPORT
A Rare Case of Heerfordt’s Syndrome with Bilateral Facial Palsy
Jamunarani Srirangaramasamy1,3 • Shakthesh Kathirvelu2
Received: 27 April 2015 / Accepted: 8 February 2016 / Published online: 19 February 2016
Ó Association of Otolaryngologists of India 2016
Abstract Sarcoidosis is a multi systemic granulomatous
disorder involving all the organs of the body. Heerfordt’s
syndrome is an acute and rare presentation of sarcoidosis. It
presents with fever, uveitis and bilateral parotid swelling
with unilateral or bilateral facial palsy. It is seen in 0.3 %
of cases of sarcoidosis. Typical presentation of Heerfordt’s
syndrome is rare. Facial palsy is seen in 25–50 % of
Heerfordt’s syndrome. Bilateral facial palsy is very rare
contributing to only 0.3–2 % of all cases of facial nerve
palsies. The diagnosis is very difficult as the facial features
are less obvious compared to unilateral palsy. In this case
report, we have presented a rare case of Heerfordt’s syn-
drome with bilateral facial palsy which was misdiagnosed
as mumps. This case highlights the importance of including
Heerfordt’s syndrome in evaluating the causes of facial
palsy. Bilateral facial palsy unlike unilateral is less obvi-
ous. Hence a detailed history, thorough clinical examina-
tion and relevant investigations will aid in arriving at
diagnosis and better management of the patient.
Keywords Bilateral facial palsy
Heerfordt’s syndrome

Neurosarcoidosis
Sarcoidosis
& Jamunarani Srirangaramasamy
jamunashakthesh@gmail.com
1
Department of Pathology, Tagore Medical College and
Hospital, Chennai, India
2
Hopkins ENT, Chennai, India
3 He was unable to blow, whistle and frown. Complete eye
37-D, Varsha Enclave, CTO Colony Second Street,
Lakshmipuram, Tambaram West, Chennai, Tamilnadu
600045, India
Introduction
Sarcoidosis is a multisystemic granulomatous disorder. The
disease has varied presentation from acute to chronic.
Heerfordt’s syndrome is an acute manifestation of sar-
coidosis seen in 0.3–1.2 % of the cases of sarcoidosis [1,
2]. It presents with fever, uveitis and bilateral parotid
swelling with facial palsy. Facial palsy is seen in 25–50 %
of the cases of Heerfordt’s syndrome [3]. Facial palsy may
be unilateral or bilateral. The differential diagnosis of
facial palsy are many. Hence a detailed history, clinical
examination and investigations are essential for diagnosis.
Here we report a rare case of Heerfordt’s syndrome with
bilateral facial palsy which was misdiagnosed as mumps.
Because most patients with Heerfordt’s syndrome present
with atypical symptoms at onset, many are misdiagnosed or
remain undiagnosed. This case highlights the importance of
knowing the rare manifestations of sarcoidosis thereby
helping the clinician arriving at the diagnosis and
treatment.
Case Report
A 32 year old male patient was referred with a provisional
diagnosis of mumps. He complained of fever, headache
and bilateral parotid swelling 1 month duration. Over the
prior 2 months he noticed a sudden onset of redness and
grittiness of both the eyes. The attacks were intermittent
and he was treated with topical steroids.
Clinical examination revealed bilateral non tender par-
otid swelling. He had an expressionless face (mask like).
closure was not possible (lagophthalmus) suggesting
bilateral facial palsy. Slit lamp examination revealed
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S1028 Indian J Otolaryngol Head Neck Surg (November 2019) 71(Suppl 2):S1027–S1029

Fig. 2 MRI thorax showing hilar and mediastinal lymphadenopathy

Fig. 1 MRI showing bilateral parotid enlargement

bilateral anterior uveitis with corneal ulceration in the right

eye. Fundus examination was normal.
Complete blood counts were normal. The serum C-re-
active protein (CRP) levels were elevated. Magnetic reso-
nance imaging (MRI) of the parotid revealed bilateral
parotid enlargement (Fig. 1). MRI of the thorax revealed
mediastinal and hilar lymphadenopathy (Fig. 2). MRI. Fine
needle aspiration biopsy (FNAB) of the parotid revealed
numerous non caseating granulomas composed of epithe-
lioid cells, Langhan’s giant cells and lymphocytes (Fig. 3).
Special stains for acid fast bacilli and fungus were nega-
tive. Serum angiotensin converting enzyme (SACE) levels
were 464 u/l (normal = 8–65 u/l).
Fig. 3 FNAB of parotid showing non caseating granuloma
The patient had fever, anterior uveitis and bilateral
parotid enlargement with bilateral facial palsy confirming
the diagnosis of Heerfordt’s syndrome. The diagnosis was characterized by fever, bilateral hilar adenopathy, erythema
supported by FNAB from the parotid, mediastinal, hilar nodosum and arthralgia.
lymphadenopathy and SACE levels. He was treated with Heerfordt’s syndrome is a rare presentation of sar-
corticosteroids. The patient had symptomatic improvement coidosis occurring only in 0.3 % of sarcoidosis [1].
with resolution of parotid swelling within a week. The According to Sharma et al. [2], in India Heerfordt’s syn-
facial palsy resolved in a month. drome is seen in 1.2 % of sarcoidosis. Heerfordt’s syn-
drome was first described in 1909 by Dr. Christian Fredrick
Heerfordt, a Danish ophthalmologist [4]. It is defined as an
Discussion atypical manifestation of sarcoidosis. It presents with a
triad of fever, uveitis and swelling of the parotid glands
Sarcoidosis is a multisystemic granulomatous disorder. The with facial palsy. Acute onset of facial palsy in Heerfordt’s
usual organs involved are the lungs and lymph nodes. syndrome is 25–50 % [3]. The facial palsy can be unilateral
However it can involve any organ of the body. The acute or bilateral. In one case initially the facial palsy was uni-
presentation of sarcoidosis occurs as Lofgren’s syndrome lateral which was misdiagnosed as Bell’s palsy [1]. Later
and Heerfordt’s syndrome. Lofgren’s syndrome is the patient developed bilateral facial palsy.

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Indian J Otolaryngol Head Neck Surg (November 2019) 71(Suppl 2):S1027–S1029
Unlike unilateral facial palsy which in most cases
idiopathic, bilateral palsy usually signifies an underlying
serious medical illness [5]. The differential diagnosis of
bilateral facial palsy include infections such as mumps,
infectious mononucleosis, HIV, Guillain–Barre syndrome
and auto immune disorders such as amyloidosis. According
to Kato et al. [6], a case of Heerfordt’s syndrome was
misdiagnosed as Guillain–Barre syndrome. Hence it is of
paramount importance to obtain a comprehensive medical
history from the patient and detailed investigations to rule
out the differential diagnosis of facial palsy.
The diagnosis of Heerfordt’s syndrome is based on a
detailed clinical history, radiological findings supported by
histologic evidence of non caseating epithelioid cell gran-
ulomas in the absence of organisms and particles [7]. The
clinical history includes fever, malaise and weight loss with
signs of lower motor neuron type of facial palsy. Clinically
the parotid glands are enlarged. Fine needle aspiration
cytology from the parotid reveal non caseating granulomas.
Special stains for acid fast bacilli and fungus are essential
to rule out other causes of granulomas [8]. Slit lamp
examination and fundoscopy are essential for eye evalua-
tion. The most common ophthal finding is anterior uveitis
seen in 65 % of cases [8]. The other findings include
posterior uveitis, pan uveitis, bilateral granulomatous
uveitis, swelling of both the eyelids and floaters.
Serum angiotensin converting enzyme (SACE)levels
will be elevated in sarcoidosis as the granulomas produce
the enzyme. SACE levels will be elevated in 60 % of
sarcoidosis. However it is neither sensitive nor specific due
to ACE gene polymorphism. Hence SACE levels can be
used in monitoring the treatment and prognosis of the
disease.
The management of Heerfordt’s syndrome is mainly
with corticosteroid therapy. In refractory cases immuno
suppressants like mycophenolate mofetil, cyclosporine and
infliximab can be used. Topical artificial tear drops can be
S1029
used since complete eye closure is not possible due to
facial palsy.
Conclusions
The symptomatology of Heerfordt’s syndrome is variable
and the disease presents a diagnostic challenge to the
clinicians. This case highlights a rare and an acute pre-
sentation of sarcoidosis which was misdiagnosed as
mumps. Hence a thorough knowledge of this entity may aid
the clinician in making the correct diagnosis and hence the
treatment.
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