Acute posterior multifocal placoid pigment epitheliopathy with concurrent cerebral vasculitis and sarcoidosis

Abstract

Dear Editor, Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a distinct clinical entity of unknown origin, characterized by the acute onset of multiple lesions at the posterior retinal pole at the pigment epithelium level. The clinical course is usually self-limited. In this report, a case of APMPPE emerging simultanously with sarcoidosis and CNS vascular abnormalities is described, suggesting that APMPPE is associated with a generalized inflammatory disease.A 40-year-old woman presented with bilateral visual field defects. Ophthalmoscopy showed multiple yellowwhite placoid lesions at the posterior pole of both eyes (Fig. 1a). Fluorescein angiography demonstrated an early hypofluorescence and a late hyperfluorescence of the lesions characteristic of APMPPE (Fig. 1b). The medical history included episodes of fever, cough and painful nodular skin lesions. Biopsy of these skin lesions demonstrated granulomatous epitheloid cells consistent with erythema nodosum. X-ray disclosed bilateral enlargement of the hilar lymph nodes, suggestive of sarcoidosis. Bronchoalveolar lavage showed a high CD4/CD8 ratio of 3.6: transbronchial biopsy revealed non-caseating granulomas (Fig. 2a), and the serum level of angiotension-converting enzyme was elevated. Cranial MRI revealed stenosis of the left middle cerebral artery (Fig. 2b).There are a few reports on associations of APMPPE with either probable sarcoidosis [1,2] or CNS abnormalities [3]. Histopathological studies have shown the latter to be caused by granulomatous arteritis of medium-sized arteries [4]. Fig. 1 Multiple yellow-white placoid lesions at the posterior pole (a), showing late hyperfluorescence in fluorescence angiography (b) V. Prokosch (*) : S. Thanos :

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“…Several authors2 3 11 12 14 reported an associated cerebral vasculitis, which could not be shown by intracerebral MR-angiography in our patient. In general, we agree with other authors,4 14 15 who suggest that an MRI should be performed in any APMPPE patient presenting with neurological symptoms.…”

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“…30 It is also known to occur in association with intraocular inflammatory disease such as acute posterior multifocal placoid pigment epitheliopathy. 31 Recently, the list of associations for erythema nodosum has been expanded to include COVID-19. 32 When associated with other eliciting conditions, erythema nodosum rep-resents an epiphenomenon that does not alter the ultimate course of the underlying disease.…”

Fatal Ischemic Stroke Complicating Acute Multifocal Placoid Pigment Epitheliopathy

“…Complaints of a "viral" prodrome with fever, malaise, upper respiratory symptoms, myalgias, or arthralgias have been reported in about 40% of cases (2,6). In a small but significant minority of patients, evidence of a systemic vasculitis exists including erythema nodosa (2,9,14). Some patients have developed AMPPE in the context of recent vaccination (13) and antibiotic administration (2).…”

“…Associated infectious agents include adenovirus (10), measles (11), and mycobacterium (12). Other systemic autoimmune or collagen vascular diseases associated with AMPPE include sarcoidosis (14,15), psoriatic arthritis (3), perinuclear antinuclear cytoplasmic antibody (ANCA)-positive systemic vasculitis (16), and central nervous system (CNS) vasculitis (17). In a small but significant minority of patients, evidence of a systemic vasculitis exists including erythema nodosa (2,9,14).…”