Heerfordt's syndrome is a rare subtype of sarcoidosis and features a combination of facial palsy, parotid swelling, and uveitis, associated with a low-grade fever. Cases with two of three symptoms are called "incomplete Heerfordt's syndrome." Heerfordt's syndrome involving other cranial nerve symptoms is relatively rare. We herein report a case of incomplete Heerfordt's syndrome presenting with trigeminal nerve palsy and a reversed halo sign, a rare manifestation of pulmonary sarcoidosis. The histological diagnosis following a biopsy of the parotid gland and endobronchial ultrasound-guided trans-bronchial needle aspiration of the mediastinal lymph nodes was sarcoidosis. The symptoms and lung lesions improved after corticosteroid therapy.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8222113PMC
http://dx.doi.org/10.2169/internalmedicine.6176-20DOI Listing

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Article Synopsis
  • Sarcoidosis is a complex disease where the immune system forms granulomas, most often affecting the lungs but can involve multiple organs, and typically responds well to glucocorticoids; however, some cases can be severe and require more aggressive treatment.
  • A case study of a 38-year-old Spanish woman with Heerfordt's syndrome showed that after initial improvement with glucocorticoids, she relapsed with significant symptoms, necessitating multiple treatments until a combination of cyclophosphamide and infliximab led to improvement.
  • Effective management of sarcoidosis, especially in severe cases, is crucial for preventing long-term damage and enhancing quality of life, with treatments tailored to the specific disease severity.
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Currently, sarcoidosis remains one of the diseases with unknown etiology, which significantly complicates its diagnosis and treatment. Various causes of sarcoidosis have been studied for many years. Both organic and inorganic trigger factors, provoking the development of granulomatous inflammation are considered.

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Multisystemic sarcoidosis revealed by a Heerfordt syndrome: case report.

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October 2022

Pneumology Department, Mohammed VI University Hospital, Faculty of Medicine and Pharmacy of Marrakech, Cadi Ayyad University, Marrakech, Morocco.

Heerfordt syndrome is a rare clinical form of sarcoidosis with a favorable evolutionary profile in the majority of cases. In its classical form, it associates uveitis, parotidomegaly, facial paralysis and fever. We report a case of multisystemic sarcoidosis type II revealed by a Heerfordt syndrome in a 51-year-old female patient.

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Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis.

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August 2021

Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Via Giustiniani 2, 35128 Padova, Italy.

Sarcoidosis is a multisystem disorder of unknown origin and poorly understood pathogenesis that predominantly affects lungs and intrathoracic lymph nodes and is characterized by the presence of noncaseating granulomatous inflammation in involved organs. The disease is highly heterogeneous and can mimic a plethora of other disorders, making diagnosis a challenge even for experienced physicians. The evolution and severity of sarcoidosis are highly variable: many patients are asymptomatic and their disease course is generally benign with spontaneous resolution.

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