| Heerfordt syndrome | |
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| Other names | Uveoparotid fever, [1] Heerfordt–Mylius syndrome, Heerfordt–Waldenström syndrome, and Waldenström's uveoparotitis [2] |
| Specialty | Angiology  |
Heerfordt syndrome is a rare manifestation of sarcoidosis. The symptoms include inflammation of the eye (uveitis), swelling of the parotid gland, chronic fever, and in some cases, palsy of the facial nerves. [1]
The exact cause of Heerfordt syndrome has not yet been definitively determined. Of those patients who have been diagnosed with Heerfordt syndrome, 15% have a close relative who also has the syndrome. One possible explanation is that the syndrome results from a combination of an environmental agent and a hereditary predisposition. Mycobacterium and Propionibacteria species have both been suggested as the environmental agent, though the evidence for this is inconclusive. [1]
In patients that have already been diagnosed with sarcoidosis, Heerfordt syndrome can be inferred from the major symptoms of the syndrome, which include parotitis, fever, facial nerve palsy and anterior uveitis. In cases of parotitis, ultrasound-guided biopsy is used to exclude the possibility of lymphoma. [3] There are many possible causes of facial nerve palsy, including Lyme disease, HIV, Melkersson–Rosenthal syndrome, schwannoma, and Bell's palsy. Heerfordt syndrome exhibits spontaneous remission.[ citation needed ]
Treatments for sarcoidosis include corticosteroids and immunosuppressive drugs. [1]
In the United States, sarcoidosis has a prevalence of approximately 10 cases per 100,000 whites and 36 cases per 100,000 blacks. [4] Heerfordt syndrome is present in 4.1 to 5.6% of those with sarcoidosis. [5]
The condition was first described in 1909 by Danish ophthalmologist Christian Frederick Heerfordt, for whom the syndrome is now named. [6] It was originally attributed to mumps, but after further studies by Swedish doctor Jan G. Waldenström in 1937, it was classified as a distinct manifestation of sarcoidosis. [2] [7]
Ramsay Hunt syndrome type 2, also known as herpes zoster oticus, is a disorder that is caused by the reactivation of varicella zoster virus in the geniculate ganglion, a nerve cell bundle of the facial nerve.
Bell's palsy is a type of facial paralysis that results in a temporary inability to control the facial muscles on the affected side of the face. Symptoms can vary from mild to severe. They may include muscle twitching, weakness, or total loss of the ability to move one, and in rare cases, both sides of the face. Other symptoms include drooping of the eyelid, a change in taste, and pain around the ear. Typically symptoms come on over 48 hours. Bell's palsy can trigger an increased sensitivity to sound known as hyperacusis.
Mumps is a viral disease caused by the mumps virus. Initial symptoms are non-specific and include fever, headache, malaise, muscle pain, and loss of appetite. These symptoms are usually followed by painful swelling of the parotid glands, called parotitis, which is the most common symptom of infection. Symptoms typically occur 16 to 18 days after exposure to the virus and resolve within two weeks. About one third of infections are asymptomatic.
Sjögren's syndrome is a long-term autoimmune disease that affects the body's moisture-producing glands, and often seriously affects other organs systems, such as the lungs, kidneys, and nervous system. Primary symptoms are dryness, pain and fatigue. Other symptoms can include dry skin, vaginal dryness, a chronic cough, numbness in the arms and legs, feeling tired, muscle and joint pains, and thyroid problems. Those affected are also at an increased risk (15%) of lymphoma.
Sarcoidosis is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly affected are the eyes, liver, heart, and brain. Any organ, however, can be affected. The signs and symptoms depend on the organ involved. Often, no, or only mild, symptoms are seen. When it affects the lungs, wheezing, coughing, shortness of breath, or chest pain may occur. Some may have Löfgren syndrome with fever, large lymph nodes, arthritis, and a rash known as erythema nodosum.
The parotid gland is a major salivary gland in many animals. In humans, the two parotid glands are present on either side of the mouth and in front of both ears. They are the largest of the salivary glands. Each parotid is wrapped around the mandibular ramus, and secretes serous saliva through the parotid duct into the mouth, to facilitate mastication and swallowing and to begin the digestion of starches. There are also two other types of salivary glands; they are submandibular and sublingual glands. Sometimes accessory parotid glands are found close to the main parotid glands.
Facial nerve paralysis is a common problem that involves the paralysis of any structures innervated by the facial nerve. The pathway of the facial nerve is long and relatively convoluted, so there are a number of causes that may result in facial nerve paralysis. The most common is Bell's palsy, a disease of unknown cause that may only be diagnosed by exclusion of identifiable serious causes.
Parotitis is an inflammation of one or both parotid glands, the major salivary glands located on either side of the face, in humans. The parotid gland is the salivary gland most commonly affected by inflammation.
Neurosarcoidosis refers to a type of sarcoidosis, a condition of unknown cause featuring granulomas in various tissues, in this type involving the central nervous system. Neurosarcoidosis can have many manifestations, but abnormalities of the cranial nerves are the most common. It may develop acutely, subacutely, and chronically. Approximately 5–10 percent of people with sarcoidosis of other organs develop central nervous system involvement. Only 1 percent of people with sarcoidosis will have neurosarcoidosis alone without involvement of any other organs. Diagnosis can be difficult, with no test apart from biopsy achieving a high accuracy rate. Treatment is with immunosuppression. The first case of sarcoidosis involving the nervous system was reported in 1905.
Pleomorphic adenoma is a common benign salivary gland neoplasm characterised by neoplastic proliferation of parenchymatous glandular cells along with myoepithelial components, having a malignant potentiality. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. It derives its name from the architectural Pleomorphism seen by light microscopy. It is also known as "Mixed tumor, salivary gland type", which refers to its dual origin from epithelial and myoepithelial elements as opposed to its pleomorphic appearance.
Benign lymphoepithelial lesion is a type of benign enlargement of the parotid and/or lacrimal glands. This pathologic state is sometimes, but not always, associated with Sjögren's syndrome.
Sialadenitis (sialoadenitis) is inflammation of salivary glands, usually the major ones, the most common being the parotid gland, followed by submandibular and sublingual glands. It should not be confused with sialadenosis (sialosis) which is a non-inflammatory enlargement of the major salivary glands.
Uveoparotitis is a symptom of sarcoidosis. It describes a chronic inflammation of the parotid gland (parotitis) and part of the eye called the uvea (uveitis). There is also a phenomenon called Waldenström's uveoparotitis, where the symptom is related to Heerfordt's syndrome. The condition was first described in 1909.
Tolosa–Hunt syndrome (THS) is a rare disorder characterized by severe and unilateral headaches with orbital pain, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles.
Salivary gland tumours also known as mucous gland adenomas or neoplasms are tumours that form in the tissues of salivary glands. The salivary glands are classified as major or minor. The major salivary glands consist of the parotid, submandibular, and sublingual glands. The minor salivary glands consist of 800-1000 small mucus-secreting glands located throughout the lining of the oral cavity. Patients with these types of tumours may be asymptomatic.
Oral and maxillofacial pathology refers to the diseases of the mouth, jaws and related structures such as salivary glands, temporomandibular joints, facial muscles and perioral skin. The mouth is an important organ with many different functions. It is also prone to a variety of medical and dental disorders.
Sarcoidosis is a systemic disease of unknown cause that results in the formation of non-caseating granulomas in multiple organs. The prevalence is higher among blacks than whites by a ratio of 20:1. Usually the disease is localized to the chest, but urogenital involvement is found in 0.2% of clinically diagnosed cases and 5% of those diagnosed at necropsy. The kidney is the most frequently affected urogenital organ, followed in men by the epididymis. Testicular sarcoidosis can present as a diffuse painless scrotal mass or can mimic acute epididymo-orchitis. Usually it appears with systemic manifestations of the disease. Since it causes occlusion and fibrosis of the ductus epididymis, fertility may be affected. On ultrasound, the hypoechogenicity and ‘infiltrative’ pattern seen in the present case are recognized features. Opinions differ on the need for histological proof, with reports of limited biopsy and frozen section, radical orchiectomy in unilateral disease and unilateral orchiectomy in bilateral disease. The peak incidence of sarcoidosis and testicular neoplasia coincide at 20–40 years and this is why most patients end up having an orchiectomy. However, testicular tumours are much more common in white men, less than 3.5% of all testicular tumours being found in black men. These racial variations justify a more conservative approach in patients of Afro-Caribbean descent with proven sarcoidosis elsewhere. Careful follow-up and ultrasonic surveillance may be preferable in certain clinical settings to biopsy and surgery, especially in patients with bilateral testicular disease.
Salivary gland diseases (SGD) are multiple and varied in cause.
A parotidectomy is the surgical excision (removal) of the parotid gland, the major and largest of the salivary glands. The procedure is most typically performed due to neoplasms (tumors), which are growths of rapidly and abnormally dividing cells. Neoplasms can be benign (non-cancerous) or malignant (cancerous). The majority of parotid gland tumors are benign, however 20% of parotid tumors are found to be malignant. Parotidectomy is performed mostly by oral and maxillofacial surgeon and ENT surgeon.
Vogt–Koyanagi–Harada disease (VKH) is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. VKH may variably also involve the inner ear, with effects on hearing, the skin and the meninges of the central nervous system.
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