Acute posterior multifocal placoid pigment epitheliopathy as the initial manifestation of sarcoidosis

Abstract

Purpose: To report an undiagnosed case of systemic sarcoidosis manifesting with bilateral acute posterior multifocal placoid pigment epitheliopathy (APMPPE).

Case report: A 26-year-old Caucasian man was referred for management of unilateral visual loss together with a paracentral scotoma developing 2 weeks after a flu-like syndrome. Clinical signs and ancillary diagnostic investigations suggested APMPPE. Laboratory tests demonstrated elevated serum angiotensin converting enzyme and lysozyme levels. Chest CT-scan disclosed moderate hilar lymph node calcifications but QuantiFERON-TB gold test was negative and bronchoalveolar lavage and biopsies were unremarkable. Accessory salivary gland biopsy disclosed epithelioid and gigantocellular granuloma formation without caseum, confirming a diagnosis of sarcoidosis. The fellow eye was involved a few days later and the patient complained of dyspnea. Echocardiography disclosed severe granulomatous myocardial infiltration and high dose corticosteroids and intravenous cyclophosphamide were initiated. Systemic treatment controlled both cardiac and ocular lesions, and was tapered accordingly.

Conclusion: The constellation of "white dot syndromes" and systemic symptoms necessitates a general work-up to exclude granulomatous disorders such as sarcoidosis or tuberculosis. Delayed diagnosis of cardiac sarcoidosis may have life-threatening consequences and the ophthalmologist may be the first physician to diagnose the condition.

Keywords: APMPPE; Dyspnea; Indocyanine Green Angiography; OCT; Sarcoidosis.

Figure 1

Fundus photograph at presentation reveals multifocal white-yellow lesions predominantly in the left eye.

Figure 2

(a) Early and (b) intermediate fluorescein angiography frames show hyperfluorescence with moderate staining in the right eye.

Figure 3

(a) Early and (b) intermediate fluorescein angiography frames show hyperfluorescence with moderate staining in the left eye.

Figure 4

(a) Early and (b) late ICGA frames reveal more extensive areas of hypofluorescence in the left eye as compared to lesions observed on the fundus photograph.

Figure 5

Spectral domain OCT shows hyper-reflectivity at the level of the RPE.