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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No rashes were noted.

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Case Reports in Otolaryngology

The condition was first described in by Danish ophthalmologist Christian Frederick Heerfordt, for whom the syndrome is now named. Progressive multifocal leukoencephalopathy developed in incomplete Heerfordt syndrome, a rare manifestation of sarcoidosis, without steroid therapy responding to cidofovir.

Oral corticosteroids represent the first line treatment option. Sarcoidosis is a systemic granulomatous disease of unknown etiology. National Center for Biotechnology InformationU. A year-old hedrfordt visited our hospital due to left facial palsy and bilateral hearing loss lasting for 4 days. The facial palsy of left side was categorized as HB grade III and bilateral sensorineural hearing loss was detected by pure tone audiometry Figure 4.

Granulomas with a peripheral lymphocyte deficit are found in the anatomic pathology of affected organs. Articles Cases Courses Quiz.

Case Reports in Otolaryngology. Typical clinical findings include cough, dyspnea, chest pain, fever, weight loss, arthralgias, and erythema nodosum. Anterior uveitis classically presents with infected conjunctiva, blurred vision, or eye pain The prognosis of sarcoidosis varies according to disease duration, severity and the organs affected.


However, the patient required urgent hospitalization for heart failure after 2 months. The constellation of symptoms along with blood samples and heerfotdt usually allows the diagnosis.

Physical examination revealed swelling of the bilateral parotid glands and her general condition was good. Fine-needle aspiration of a left preauricular lymph node revealed multinucleated giant cells and noncaseating granulomas consistent with granulomatous lymphangitis Figure 2.

Computed tomography CT of the chest showed hilar lymphadenopathy with bibasilar interstitial lung disease. Preauricular lymph node biopsy showing herrfordt noncaseating granuloma.

[Heerfordt syndrome].

Although initial response rates to corticosteroids are high, a relapse of the symptoms may occur during the tapering of the corticosteroid dose. Radiologic manifestations of sarcoidosis in various organs. Positron emission tomography PET showed hypermetabolic activity in the right parotid gland, right eyelid, anterior mediastinal lymph nodes, groin lymph nodes, and subcutaneous nodule in the thigh Figure 2. Loading Stack – 0 images remaining. This article has been cited by other articles in PMC.

After three months, the patient developed a cough, chest pain and dyspnea and was admitted for investigation. No nodules were detected and diffuse swelling of parotid gland was observed in the US examination. Over the prior 4 months, she had had a productive cough, fevers, night sweats, and an unintentional pound weight loss. Treatment with a heertordt steroid improved her parotid gland enlargement, facial palsy, and anterior uveitis.

Cases with loss of taste and hyperacusis have also been reported [ 7 ]. View at Google Scholar T. Her serum angiotensin-converting enzyme level was twice the upper limit of normal. She was unable to close her left eyelid completely. Find articles by Priscila Kakizaki. Heerfordt syndrome Dr Rohit Sharma and A. This ssyndrome had marked improvement of symptoms after 4 days of prednisone therapy. J Natl Med Assoc. Further, there is a possibility that epineurial necrotizing vasculitis could also lead to nerve ischemia with subsequent axonal degeneration [ 13 ].


Three patients had the full form, 13 had the incomplete form, and all had uveitis. Nerve granulomas and vasculitis in sarcoid peripheral neuropathy: Infobox medical condition new. As a result of consultation with the Department of Respiratory Medicine, treatment with prednisolone was started based on the premise that there was a possibility of a delayed histological confirmation of the diagnosis.

Author information Article notes Copyright and License information Disclaimer. Journal List An Bras Dermatol sndrome. As the prognosis for facial nerve palsy in sarcoidosis is thought to be good [ 1415 ], detailed examination, including bronchoscopic biopsy, should proceed in advance of the treatment for facial nerve palsy.

Chest tomography revealed a large number of small bilateral parenchymal nodules and intense mediastinal adenomegaly. Support Synerome Support Center. Electrophysiological tests, including electroneurography ENoG and the nerve excitability test NETshowed no severe damage to the facial nerve, which indicated a good prognosis for the palsy.