Abstract

Heerfordt's syndrome is a rare manifestation of sarcoidosis characterized by the presence of facial nerve palsy, parotid gland enlargement, anterior uveitis, and low grade fever. Two cases of Heerfordt's syndrome and a literature review are presented. Case 1. A 53-year-old man presented with swelling of his right eyelid, right facial nerve palsy, and swelling of his right parotid gland. A biopsy specimen from the swollen eyelid indicated sarcoidosis and he was diagnosed with incomplete Heerfordt's syndrome based on the absence of uveitis. His symptoms were improved by corticosteroid therapy. Case 2. A 55-year-old woman presented with left facial nerve palsy, bilateral hearing loss, and swelling of her bilateral parotid glands. She had been previously diagnosed with uveitis and bilateral hilar lymphadenopathy. Although no histological confirmation was performed, she was diagnosed with complete Heerfordt's syndrome on the basis of her clinical symptoms. Swelling of the bilateral parotid glands and left facial nerve palsy were improved immediately by corticosteroid therapy. Sarcoidosis is a relatively uncommon disease for the otolaryngologist. However, the otolaryngologist may encounter Heerfordt's syndrome as this syndrome presents with facial nerve palsy and swelling of the parotid gland. Therefore, we otolaryngologists should diagnose and treat Heerfordt's syndrome appropriately in cooperation with pneumologists and ophthalmologists.

Highlights

  • Sarcoidosis is a systemic granulomatous disease of unknown etiology

  • Heerfordt’s syndrome is characterized by the presence of facial nerve palsy, parotid gland enlargement, anterior uveitis, and low grade fever [1]. This syndrome is considered as a subtype of sarcoidosis, with the complete form of Heerfordt’s syndrome constituting only 0.3% of all cases of sarcoidosis [3]

  • According to the diagnostic guidelines for sarcoidosis proposed by the Japan Society of Sarcoidosis and other Granulomatous Disorders in 2006 [8], Heerfordt’s syndrome is classified into complete type, in which all four main symptoms are presented, and incomplete type, in which two out of the three symptoms of facial nerve palsy, parotid gland enlargement, and anterior uveitis are detected

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Summary

Introduction

Sarcoidosis is a systemic granulomatous disease of unknown etiology. It usually affects the lung, any organ may be involved. Heerfordt’s syndrome is a rare manifestation of sarcoidosis characterized by the presence of facial nerve palsy, parotid gland enlargement, anterior uveitis, and low grade fever [1]. Heerfordt described three patients with uveitis, parotid swelling, cranial nerve palsy, and fever in 1909 [1] and Waldenstrom classified it as a distinct manifestation of sarcoidosis in 1937 [2]. The simultaneous presence of all symptoms represents the complete form of this syndrome, with the complete form constituting 0.3% of all cases of sarcoidosis [3].

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