Case 1. 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. Case 2. A year-old woman presented with left facial nerve palsy, bilateral hearing loss, and swelling of her bilateral parotid glands.
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Evidence of recent weight loss. Facial swelling around the cheeks. Swollen eyelids. Inflamed eyes, squinting in light.
Visible facial nerve palsy with drooping of features on one side NB: if bilateral, facial symmetry may be preserved. Examine other cranial nerves.
Bilateral lower motor neurone facial nerve palsy of abrupt onset in young adults is virtually always due to sarcoidosis. There may be accompanying signs of meningism. Eyes Anterior uveitis is painful and presents with: Miosis. Injected conjunctivae next to the cornea so-called perilimbal flush. Fundoscopy may show retinal detachment or vasculitis and papilloedema if there is posterior uveitis.
Severe cases may show hypopyon - a collection of yellowish inflammatory exudate and cells at the bottom of the anterior chamber. Thoroughly examine the heart, chest, skin, eyes, joints, abdomen, lymph fields, nervous system, salivary glands and upper respiratory tract in all patients presenting with suspected sarcoidosis in order to detect the full extent of the disease.
Differential diagnosis The syndrome is a fairly distinct clinical entity and once recognised is not likely to be confused with illnesses other than sarcoidosis, being virtually pathognomonic for the disease. Sarcoidosis has a vast differential diagnosis, depending on its mode of presentation.
See also the separate Uveitis and Facial Nerve Palsy articles. Investigations Serum angiotensin-converting enzyme ACE - which is usually elevated. Gallium scanning of the parotid gland may be helpful. Biopsy of the parotid gland and other sites of the disease may be required in some cases and may reveal the classic non-caseating epithelioid granulomata.
Optic nerve involvement may be detected by imaging - MRI is of more assistance than CT in this situation. Additional investigations may include: CXR - to look for evidence of hilar adenopathy or pulmonary involvement. Autoantibodies if there is any suspicion of a connective tissue disease. Lumbar puncture, which may be needed where there is suspicion of meningitis - in sarcoidosis, it shows a sterile pleomorphic inflammatory picture.
The disease course is very variable and difficult to predict. Medical The most troublesome aspect of the syndrome to manage is uveitis which may be sight-threatening and requires expert ophthalmological assessment and monitoring. Topical or systemic corticosteroids are the mainstays of management of ocular sarcoidosis.
Other immunomodulatory drugs may be used as steroid-sparing or disease-modifying agents. Systemic steroids are used to treat the facial nerve palsy if it is troublesome enough and does not resolve - usually successfully. Surgical Surgical intervention may be used to treat the complications of cataracts or vitreous opacification, once active disease has been controlled. Uveitis increases the risk of glaucoma, so a significant proportion of patients may require trabeculectomy or other glaucoma drainage devices.
Two Cases of Heerfordt’s Syndrome: A Rare Manifestation of Sarcoidosis
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