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.

Keywords: central nervous system vasculitis; digital subtraction angiography; internal carotid artery occlusion; lupus pernio; neurosarcoidosis; non-caseating granuloma; sarcoidosis.

Figure 1. Axial MR images depicting microangiopathic ischemic changes consistent with small vessel pathology

Multiple foci are noted distributed bilaterally in the frontal regions. (A) Foci appear hypointense on axial T1-weighted MR image (red arrow). (B and C) Same foci appear hyperintense on axial T2-weighted and FLAIR MR images (white arrows). (D) GRE sequence does not show any bleed. MR, magnetic resonance; FLAIR, fluid-attenuated inversion recovery; GRE, gradient-echo.

Figure 2. Axial MR images depicting microangiopathic ischemic changes consistent with small vessel pathology

Multiple foci are noted distributed bilaterally in the frontoparietal regions and centrum semiovale. (A) Foci appear hypointense on axial T1-weighted MR image (red arrows). (B and C) Same foci appear hyperintense on axial T2-weighted and FLAIR MR images (white arrows). (D) GRE sequence does not show any bleed. MR, magnetic resonance; FLAIR, fluid-attenuated inversion recovery; GRE, gradient-echo.

Figure 3. Axial MR images showing DW and ADC sequences

Multiple foci are noted distributed bilaterally in the frontoparietal regions. No diffusion restriction is demonstrated on (A) axial DW MR image and (B) axial ADC MR image (white arrows). MR, magnetic resonance; DW, diffusion-weighted; ADC, apparent diffusion coefficient.

Figure 4. Right carotid artery angiogram AP view on DSA brain

Position of catheter is visible in right common carotid artery (thin black arrow). Complete cut-off of contrast flow is noted at origin of right internal carotid artery (thick white arrow); however, contrast flow is diverted to right external carotid artery (thick black arrow). DSA, digital subtraction arteriography; AP, anteroposterior.

Figure 5. Left vertebral artery angiogram AP view on DSA brain

Normal contrast flow in the left vertebral artery is visible (thick black arrow); however, basilar artery through out its course is attenuated with reduced contrast flow (thick white arrow). DSA, digital subtraction arteriography; AP, anteroposterior.

Figure 6. Left vertebral artery angiogram lateral view on DSA brain

Right middle cerebral artery with its branches (thick black arrow) and right anterior cerebral artery with its branches (thick white arrow) are being filled with retrograde contrast flow from right posterior communicating artery (thin black arrow). DSA, digital subtraction arteriography.

Figure 7. Lateral view showing branches of MCA and ACA on DSA brain

MCA and ACA are being supplied from posterior communicating artery on left vertebral artery angiogram. Multifocal areas of narrowing (thin black arrows) and dilatation (thin white arrows) are seen in the distal branches of MCA and ACA. This being consistent with findings of vasculitis. DSA, digital subtraction angiography; MCA, middle cerebral artery; ACA, anterior cerebral artery.