Heerfordt-Waldenström syndrome is a rare subacute variant of sarcoidosis, characterized by enlargement of the parotid or salivary glands, facial nerve paralysis. Images in Clinical Medicine from The New England Journal of Medicine — Heerfordt’s Syndrome, or Uveoparotid Fever. Heerfordt’s syndrome is a rare manifestation of sarcoidosis characterized by the presence of facial nerve palsy, parotid gland enlargement.
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This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use, distribution, and heerfofdt in any medium, provided the original work is properly cited. A year-old man presented with swelling of his right eyelid, right facial nerve palsy, and swelling of his right parotid gland.
His symptoms were improved by corticosteroid therapy. A year-old woman presented with left facial nerve palsy, bilateral hearing loss, and swelling of her bilateral parotid glands. She had been previously diagnosed hesrfordt uveitis and bilateral hilar lymphadenopathy. Swelling of the bilateral parotid glands and left facial nerve palsy were improved immediately by corticosteroid therapy. Sarcoidosis is a relatively uncommon disease for the otolaryngologist.
Sarcoidosis is a systemic granulomatous disease of unknown etiology. Although it usually affects the lung, any organ may be involved. The simultaneous presence of all symptoms represents the complete form of this syndrome, with the complete form constituting 0. A year-old man presented with swelling of his right eyelid in March and was referred to the Department of Dermatology in a local hospital.
A biopsy specimen from his eyelid revealed granulomatous blepharitis, and a granulomatous disease such as sarcoidosis was suspected. In spite of detailed examination, no uveitis or bilateral hilar heerfordt BHL was detected and he was followed up closely without treatment.
He was admitted to the Department of Dermatology in our hospital in August, because of worsening swelling of his right eyelid. As he also presented with a 3-month history of swelling of the right parotid gland and a 1-month history of right facial palsy, he was referred to the Department of Otolaryngology.
Physical examination revealed a swollen right eyelid and right parotid gland. He presented with a regular general status. A pure tone audiogram showed normal hearing and stapedial reflex on ysndrome right side was normal. The patient did not complain of impaired taste. Ultrasound US examination showed an enlarged right parotid gland interspersed with hypoechoic areas Figure 1.
Electrophysiological tests, including electroneurography ENoG and the nerve excitability test NETshowed no severe damage to the facial nerve, which indicated a good prognosis for the palsy.
Positron emission tomography PET showed hypermetabolic activity in the right parotid gland, right eyelid, anterior mediastinal lymph nodes, groin lymph nodes, and subcutaneous nodule in the thigh Figure 2. Although uveitis was not detected in the ophthalmologic examination, biopsy specimens from the right eyelid and the lymph node in the left thigh revealed noncaseating epithelioid cell granuloma and a diagnosis of sarcoidosis was made histologically Figure 3.
Cardiac sarcoidosis was also suspected on the basis of electrocardiogram results showing premature ventricular contraction. Asymmetry of the forehead continued for a few months, while the right facial nerve palsy gradually improved and was resolved completely at 4 months. Prednisolone was tapered carefully and stopped after 2 years. No recurrence has been observed to date. A year-old woman visited our hospital due to left facial palsy and bilateral hearing loss lasting for 4 days.
She had a history of postherpetic neuralgia and purpura pigmentosa chronica. As uveitis and BHL had been previously diagnosed, she underwent a detailed examination for sarcoidosis in the Department of Respiratory Medicine in our hospital. Physical examination revealed swelling of the bilateral parotid glands and her general condition was good. No nodules were detected and diffuse swelling of parotid gland was observed in the US examination. The facial palsy of left side was categorized as HB grade III and bilateral sensorineural hearing loss was detected by pure tone audiometry Figure 4.
Although geotropic direction-changing positional nystagmus was observed, no canal paresis was revealed by caloric test.
The patient was negative bilaterally for stapedial reflex. NET showed no severe damage to the facial nerve.
PET showed hypermetabolic activity in the bilateral hilar lymph nodes, mediastinal lymph nodes, lung, spleen, and myocardium Figure 5. Magnetic resonance imaging with gadolinium enhancement revealed no lesions in her brain.
Serum ACE and sIL-2R levels were elevated; however, there was no significant elevation in serum antibody for varicella zoster virus. As a result of consultation with the Department of Respiratory Medicine, treatment with prednisolone was started based on the premise that heeffordt was a possibility of a delayed histological confirmation of the diagnosis.
The swelling of the heerforrt glands improved immediately and the facial nerve palsy was resolved two weeks after the treatment. However, the patient required urgent hospitalization for heart failure after 2 months.
Cardiac sarcoidosis was strongly suspected as the cause of the heart failure. Corticosteroids are thought to be the first-choice treatment in the management of neurosarcoidosis in order to suppress inflammation, although prospective, double-blinded clinical trials have not yet been administered [ 9 — 11 ].
Although initial response rates to corticosteroids are high, a relapse of the symptoms may occur during the tapering of the corticosteroid dose. In these cases, immunosuppressant agents, including azathioprine, methotrexate, cyclosporine A, and cyclophosphamide, are used in combination with the corticosteroids [ 910 ]. Cranial nerve palsy in neurosarcoidosis may be caused by nerve granulomas, perineural inflammatory infiltrates, eherfordt cranial pressure, or granulomatous basal meningitis [ 1112 ].
Further, there is a possibility that epineurial necrotizing vasculitis could also lead to nerve ischemia with subsequent axonal degeneration [ 13 ]. Facial nerve palsy is thought to be the result of direct involvement of the facial nerve branches by the parotid lesion [ 14 ]. Cases with loss of taste and hyperacusis have also been reported [ 7 ]. The presence of these cases implies the site of the facial nerve lesion is not limited to the parotid gland.
Treatment for the facial nerve palsy was given priority over the precise diagnosis of sarcoidosis after consultation with the Department of Respiratory Medicine. However, due to syjdrome delay in the histological confirmation of sarcoidosis, deterioration of the underlying cardiac sarcoidosis resulted in heart failure.
As the prognosis for facial nerve palsy in sarcoidosis is thought to be good [ 1415 ], detailed examination, including bronchoscopic biopsy, should proceed in advance of the treatment for facial nerve palsy.
The authors declare that there is no conflict of interests regarding the publication of this paper. Case Reports in Otolaryngology.
The Heerfordt-Waldenström syndrome as an initial presentation of sarcoidosis
Indexed in Web of Science. Subscribe to Table of Contents Alerts. Table of Contents Alerts. Introduction Sarcoidosis is a systemic granulomatous disease of unknown etiology. View at Google Scholar J. View at Google Scholar T.
View at Google Scholar B.