Heerfordt syndrome

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]

Heerfordt syndrome
Other names	Uveoparotid fever,^[1] Heerfordt–Mylius syndrome, Heerfordt–Waldenström syndrome, and Waldenström's uveoparotitis^[2]
Specialty	Angiology

Causes

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]

Diagnosis

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]}

Treatment

Treatments for sarcoidosis include corticosteroids and immunosuppressive drugs.^[1]

Prevalence

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]

History

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]

See also

• Darier–Roussy disease
• Sarcoidosis
• List of cutaneous conditions

Notes

• Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.

References

1. Evanchan, Jason; Barreiro, Timothy J.; Gemmel, David (May 2010). "Uveitis, salivary gland swelling, and facial nerve palsy in a febrile woman". Journal of the American Academy of Physician Assistants. 23 (5): 46–50. doi:10.1097/01720610-201005000-00012. PMID 20480871.
2. synd/3546 at Who Named It?
3. Fischer, T.; et al. (January 2002). "Diagnosis of Heerfordt's syndrome by state-of-the-art ultrasound in combination with parotid biopsy: a case report". European Radiology. 12 (1): 134–7. doi:10.1007/s003300100879. PMID 11868089. S2CID 1088521.
4. Iannuzzi, Michael C.; Rybicki, Benjamin A.; Teirstein, Alvin S. (22 November 2007). "Sarcoidosis". New England Journal of Medicine. 357 (21): 2153–65. doi:10.1056/NEJMra071714. PMID 18032765.
5. Fukuhara K, Fukuhara A, et al. (August 2013). "Radiculopathy in patients with Heerfordt's syndrome: two case presentations and review of the literature". Brain and Nerve. 65 (8): 989–92. PMID 23917502.
6. Heerfordt C. F. (1909). "Über eine "Febris uveo-parotidea subchronica" an der Glandula parotis und der Uvea des Auges lokalisiert und häufug mit Paresen cerebrospinaler Nerven kompliziert". Albrecht von Graefes Archiv für Ophthalmologie. 70 (2): 254–273. doi:10.1007/bf02008817. S2CID 10880812.
7. Waldenström, J. G. (1937). "Some observations on uveoparotitis and allied conditions with special reference to the symptoms from the nervous system". Acta Medica Scandinavica. 91 (1–2): 53–68. doi:10.1111/j.0954-6820.1937.tb16029.x.

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