A Rare Form of Sarcoidosis: Heerfordt Syndrome

Öz

Sarcoidosis is a granulomatous disease of unknown cause, which mostly affects the lungs but can occasionally involve other systems and organs. Heerfordt syndrome (HS) is a rare form of sarcoidosis, a type of neurosarcoidosis that consists of a combination of fever, parotid gland enlargement, anterior uveitis, and facial nerve palsy. The presence of all of these symptoms is called the complete form, and the presence of two or three together is called the incomplete form. It is estimated that HS accounts for approximately 4-5% of all sarcoidosis cases. It is thought that facial paralysis may be due to enlargement of the parotid gland or direct compression of the nerve by a lesion within the facial canal. Although HS is a rare form of neurosarcoidosis, it should be considered in the differential diagnosis of facial nerve paralysis. Sarcoidosis/HS should be kept in mind in cases of unilateral facial paralysis and further examinations should be performed to diagnose HS. In this article, a case of a 45-year-old woman who was diagnosed with HS by holistic evaluation with physical examination, radiological imaging, biochemical tests and Endobronchial Ultrasonography-Transbronchial needle aspiration (EBUS-TBNA) for histopathological diagnosis is shared with the literature.

Anahtar Kelimeler

• Facial paralysis
• Heerfordt Syndrome
• Sarcoidosis

Sarkoidozun nadir bir formu; Heerfordt Sendromu

Öz

Sarkoidoz nedeni bilinmeyen, çoğunlukla akciğerleri tutan fakat zaman zaman diğer sistem ve organ tutulumu da yapabilen granülomatöz bir hastalıktır. Heerfordt sendromu (HS), sarkoidozun nadir bir fenotipi olup ateş, parotis bezinde büyüme, anterior üveit ve fasiyal sinir felcinin kombinasyonundan oluşan bir nörosarkoidoz formudur. Bu belirtilerin tümünün görülmesi tam form, iki veya üç tanesinin birlikte görülmesi eksik form olarak adlandırılır. HS’nin tüm sarkoidoz vakalarının yaklaşık %4-5’inde oluşturduğu tahmin edilmektedir. Fasial paralizi parotis bezinin büyümesine ya da fasiyal kanal içinde bir lezyonun sinire doğrudan basısına bağlı olabileceği düşünülmektedir. HS nörosarkoidozun nadir bir formu olmakla birlikte, fasiyal sinir paralizisi ayırıcı tanısında akla gelmelidir. Tek taraflı yüz felci olgularında sarkoidoz/HS akılda tutulmalı HS tanısı için ileri tetkikler yapılmalıdır. Bu yazıda 45 yaşında kadın, yapılan fizik muayene, radyolojik görüntüleme, biyokimyasal tetkikler ve histopatolojik tanıya yönelik yapılan Endobronşial Ultrasonografi-Transbronşial iğne aspirasyonu (EBUS-TBNA) ile bütüncül değerlendirilerek HS tanısı alan bir olgu literatür eşliğinde paylaşılmıştır.

Anahtar Kelimeler

• Fasiyal paralizi
• Heerfordt sendromu (HS)
• Sarkoidoz.

Kaynakça

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