Heerfordt's syndrome

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Heerfordt's syndrome
Classification and external resources
DiseasesDB 33567
MeSH D014608

Heerfordt's syndrome, also referred to as uveoparotid fever,[1] Heerfordt-Mylius syndrome, Heerfordt-Waldenström syndrome, and Waldenström’s uveoparotitis,[2] 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]

History[edit]

The condition was first described in 1909 by Danish ophthalmologist Christian Frederick Heerfordt, for whom the syndrome is now named.[3] 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][4]

Causes[edit]

The exact cause of Heerfordt's syndrome has not yet been definitively determined. Of those patients who have been diagnosed with Heerfordt's 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]

Prevalence[edit]

In the United States, sarcoidosis has a prevalence of approximately 10 cases per 100,000 whites and 36 cases per 100,000 blacks.[5] Heerfordt's syndrome is present in approximately 0.3% of those with sarcoidosis.[6]

Diagnosis and treatment[edit]

In patients that have already been diagnosed with sarcoidosis, Heerfordt's syndrome can be inferred from the major symptoms of the syndrome, which include parotitis, fever, and facial nerve palsy. In cases of parotitis, ultrasound-guided biopsy is used to exclude the possibility of lymphoma.[7] There are many possible causes of facial nerve palsy, including Lyme disease, HIV, Melkersson–Rosenthal syndrome, schwannoma, and Bell's palsy. Heerfordt's syndrome exhibits spontaneous remission. Treatments for sarcoidosis include corticosteroids and immunosuppressive drugs.[1]

See also[edit]

Notes[edit]

  • Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0. 

References[edit]

  1. ^ a b c d 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. ^ a b synd/3546 at Who Named It?
  3. ^ 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 Grafes Archiv für Ophthalmologie 70: 254–273. doi:10.1007/bf02008817. 
  4. ^ 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: 53–68. doi:10.1111/j.0954-6820.1937.tb16029.x. 
  5. ^ 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. 
  6. ^ Sugawara, Yoshifumi et al (November 2005). "Heerfordt Syndrome Initially Presenting With Subcutaneous Mass Lesions: Usefulness of Gallium-67 Scans Before and After Treatment". Clinical Nuclear Medicine 30 (11): 732–3. doi:10.1097/01.rlu.0000182264.76461.74. 
  7. ^ 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.