Central Nervous System Vasculitis Secondary to Sarcoidosis: A Rare Case of Lupus Pernio With Complete Occlusion of Right Internal Carotid Artery

Abstract

Sarcoidosis is a systemic inflammatory disorder resulting from an inappropriate immune response to ubiquitous environmental stimuli. It has a predilection for African Americans and people of Northern European countries. The classic histology is that of a non-caseating granuloma. Central nervous system involvement is a rare occurrence in sarcoidosis and even in this manifestation, the presence of vasculitis is comparatively uncommon. We present a case of a 35-year-old female, who presented with complaints of persistent headache of moderate intensity and had a violaceous plaque on nose, being treated by a dermatologist. The patient on further workup had mildly raised proteins on cerebrospinal fluid analysis. MRI brain showed multiple foci in bilateral frontoparietal regions and centrum semiovale, while digital subtraction angiography brain depicted vasculitis of small vessels of brain and complete occlusion of right internal carotid artery at its origin. Biopsy of lesion on nose was performed that showed chronic granulomatous inflammation. A diagnosis of brain vasculitis secondary to sarcoidosis was made. The patient was treated with plasmapheresis and pulse steroid therapy initially, and later on with cyclophosphamide and azathioprine. This resulted in resolution of headache and nose lesion. Copyright © 2020, Arif et al.