2024 Volume 117 Issue 11 Pages 1011-1017
Heerfordt syndrome is a rare phenotype of sarcoidosis characterized by uveitis, parotid swelling, facial nerve paralysis, and fever that accounts for less than 2% of all cases of sarcoidosis. A 56-year-old woman who had been diagnosed as having uveitis approximately four months earlier based on the presence of conjunctival hyperemia, ocular pain, and photophobia presented our department of otorhinolaryngology with right facial nerve paralysis. She had previously presented with various symptoms such as fever, arthralgia, edema and pain in both lower limbs and facial swelling. The degree of right facial nerve paralysis at her initial visit to our department was mild (score on the Yanagihara grading system, 22), and we treated with corticosteroids under the assumption of Bell’s palsy. However, she showed little improvement. Subsequently, histopathologic examination of a biopsy specimen obtained from the parotid gland showed nondesmoplastic epithelioid cell granulomas, which led to the diagnosis of sarcoidosis.
Sarcoidosis is a systemic disease characterized histologically by the presence of nondesmoplastic epithelioid cell granulomas, and the most commonly affected organs are the hilar/mediastinal lymph nodes, lungs, eyes, heart, and skin. In patients presenting with facial nerve paralysis, which is a frequently encountered condition in daily clinical practice, the possibility of an underlying systemic disease, such as sarcoidosis, should be considered in patients showing an atypical course and heterogeneous manifestations other than facial nerve paralysis, as in the present case.
