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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Sarcoidosis is a granulomatous disease of unclear etiology, which commonly presents with cough, dyspnea, chest pain, fever, weight loss, arthralgias, and erythema nodosum. Here we present a case of a year-old nonsmoking African American woman who presented with 3 days of progressively worsening left facial droop, difficulty swallowing, and blurred vision.
Over the prior 4 months, she had had a productive cough, fevers, night sweats, and an unintentional pound weight loss. Physical examination revealed a left facial droop involving the forehead, cheek, and chin with an inability to close the left eyelid.
Her serum angiotensin-converting enzyme level was twice the upper limit of normal. Prominent hilar markings were identified on chest x-ray, but no focal opacity was seen.
Fine-needle aspiration of a preauricular lymph node revealed noncaseating granulomas consistent with granulomatous lymphangitis. Treatment with a high-dose steroid improved her parotid gland enlargement, facial palsy, and anterior uveitis. A year-old nonsmoking African American woman presented with a 3-day history heeerfordt progressively worsening left facial droop, difficulty swallowing, and blurred vision. Physical examination revealed a swollen left cheek and left facial droop involving the forehead, cheek, and chin.
She was unable to close her left eyelid completely. Her posterior pharynx was erythematous, but no areas of oropharyngeal fluctuance were noted. Tender, syndroms submandibular and cervical lymph nodes were palpable bilaterally, but more prominently on the left.
Chest, lung, abdominal, and extremity exams were unremarkable. No rashes were noted. The remainder of the neurologic exam was normal. Blood cultures, tuberculosis testing, and HIV antibody testing were negative.
Antinuclear antibody and extractable nuclear antigen profiles were within normal limits. Serum angiotensin-converting enzyme ACE levels were twice the upper limit of normal.
Rheumatoid factor was not checked on initial presentation. Computed tomography CT of the chest showed hilar lymphadenopathy with bibasilar interstitial lung disease.
CT of the neck soft tissues demonstrated enlarged lymph nodes, the largest of which measured 3. Esophagogastroduodenoscopy showed pharyngeal edema but no evidence of obstruction. The patient’s ophthalmologic exam was notable for impaired corneal sensation, punctate epithelial erosions in both eyes, and no overt flare or leukocytes seen on slit lamp exam. Fundi were normal in appearance.
Fine-needle aspiration of a left preauricular lymph node revealed multinucleated giant cells and noncaseating granulomas consistent with granulomatous lymphangitis Figure 2. No bacteria or viruses were syndromr on culture of the biopsied lymph node.
Given the patient’s acute onset of unilateral facial droop, the differential diagnosis includes vascular, infectious, granulomatous, neoplastic, and autoimmune causes of cranial nerve VII injury Table 1.
The principal vascular causes of unilateral facial weakness are ischemic stroke and intraparenchymal hemorrhage involving the cranial nerve VII nucleus.
Infectious causes of unilateral facial palsy include herpes simplex virus—associated Bell’s palsy, Lyme disease, HIV, and varicella zoster virus—associated Ramsay-Hunt syndrome. Tuberculosis, orofacial granulomatosis, and sarcoidosis are granulomatous diseases that cause unilateral facial palsy.
Do you know this syndrome? Heerfordt-Waldenström syndrome
In tuberculosis, the mastoid, middle ear, or petrous bone is often involved 12. Sarcoidosis is a systemic disease characterized by granuloma formation. Typical clinical findings include cough, dyspnea, chest pain, fever, weight loss, arthralgias, and erythema nodosum.
Christian Heerfordt first described this constellation of symptoms in The facial nerve palsy is associated with epineural granulomas and perineural inflammatory infiltrates of cranial nerve VII Anterior uveitis classically presents with infected conjunctiva, blurred vision, or eye pain This patient’s blurred vision and eye pain were thought to be related to uveitis.
The patient had already received high-dose steroids for 3 days at the time of the full ophthalmologic exam, which may explain why the slit lamp and funduscopic exams were fairly unremarkable. Salivary gland involvement likely led to the pharyngitis seen on esophagogastroduodenoscopy and the patient’s difficulty swallowing.
Granulomas in sarcoidosis are known to produce ACE; however, the serum ACE level does not always correlate precisely with disease activity 14 As with other manifestations of sarcoidosis, corticosteroids are the mainstay of treatment. Immunosuppressants such as mycophenolate mofetil, cyclosporine, and infliximab can be utilized in patients whose symptoms are refractory to steroids 16 When complete eye closure is not possible, the patient should use artificial tears while awake, use lubricant eye ointment at night, and cover the affected eye during sleep to prevent exposure keratopathy.
If the facial nerve palsy is not treated with steroids, the likelihood of permanent facial paralysis is increased. When the facial paralysis is misdiagnosed as being a simple Bell’s palsy, the opportunity to treat the systemic sarcoidosis is missed. This patient had marked improvement of symptoms after 4 days of prednisone therapy.
Two months after discharge, her facial nerve palsy, parotitis, fevers, and uveitis had resolved.
We would like to acknowledge Adrian J. National Center for Biotechnology InformationU. Proc Bayl Univ Med Cent. Author information Copyright and License information Disclaimer. This article has been cited by other articles in PMC. Abstract Sarcoidosis is synrdome granulomatous disease of unclear etiology, which commonly presents with cough, dyspnea, chest pain, fever, weight loss, arthralgias, and erythema nodosum.
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CT of the neck soft tissues demonstrating prominent cervical lymphadenopathy. Preauricular lymph node biopsy showing a noncaseating granuloma. Etiologies of facial nerve palsy. Acknowledgments We would like to acknowledge Adrian J. Adams and Victor’s Principles of Neurology.
Orofacial granulomatosis—a year review. N Engl J Med.
Two Cases of Heerfordt’s Syndrome: A Rare Manifestation of Sarcoidosis
Sarcoidosis and its neurological manifestations. Orofacial presentations of sarcoidosis—a sydnrome series and review of the literature. Heerfordt syndrome with unilateral facial nerve palsy: Uveitis, salivary gland swelling, and facial nerve palsy in a febrile woman. J Natl Med Assoc. Some observations of uveoparotitis and allied conditions with special reference to the symptoms from the nervous system.
Nerve granulomas and vasculitis in dyndrome peripheral neuropathy: Current approach in diagnosis and management of anterior uveitis. Angiotensin I-converting enzyme ACE for sarcoidosis diagnosis.