The Heerfordt-Waldenström syndrome as an initial presentation of sarcoidosis

Abstract

Sarcoidosis is a granulomatous disease of unclear etiology, which commonly presents with cough, dyspnea, chest pain, fever, weight loss, arthralgias, and erythema nodosum. Heerfordt-Waldenström syndrome, a rare presentation of sarcoidosis, is characterized by the presence of parotid gland enlargement, facial palsy, anterior uveitis, and fever. Here we present a case of a 59-year-old nonsmoking African American woman who presented with 3 days of progressively worsening left facial droop, difficulty swallowing, and blurred vision. Over the prior 4 months, she had had a productive cough, fevers, night sweats, and an unintentional 30-pound weight loss. Physical examination revealed a left facial droop involving the forehead, cheek, and chin with an inability to close the left eyelid. Her serum angiotensin-converting enzyme level was twice the upper limit of normal. Prominent hilar markings were identified on chest x-ray, but no focal opacity was seen. Fine-needle aspiration of a preauricular lymph node revealed noncaseating granulomas consistent with granulomatous lymphangitis. The patient was given a diagnosis of Heerfordt-Waldenström syndrome, or uveoparotid fever. Treatment with a high-dose steroid improved her parotid gland enlargement, facial palsy, and anterior uveitis.

Figure 1.

CT of the neck soft tissues demonstrating prominent cervical lymphadenopathy.

Figure 2.

Preauricular lymph node biopsy showing a noncaseating granuloma.