An eosinophilic variant granulomatosis with polyangiitis involving the dura, bilateral orbits, and mastoids

Photograph showing: A) Coronal high resolution T2 weighted image (1) demonstrating increased signal intensity in the soft tissue along lateral aspect of the left orbit. T1 weighted image (2) shows corresponding decreased signal intensity. Post contrast T1 weighted images (3,4) show enhancement in the abnormal orbital soft tissue. Minimal similar abnormalities are seen in the right orbit as well. There is also abnormal meningeal thickening and enhancement along the base and lateral aspect of the cerebral hemisphere. B) Axial post contrast T1 weighted images (5,6) show enhancement of the abnormal soft tissue as well as abnormal thickening and enhancement of the dura on left side. Axial pre contrast T1 weighted image (7) shows ill-defined soft tissue thickening around the lateral aspect of the left orbit.

Photograph showing: A) Lacrimal gland biopsy shows granulation, medium sized vessel wall infiltrated by eosinophils (arrow head) and plasma cells. The lumen of the vessel is obliterated by similar cells and there are newly formed vessels (hematoxylin and eosin stain X400). B) Lacrimal gland biopsy shows eosinophilic granuloma. The arrow head points to an aggregate of histiocytes. There are numerous plasma cells at the periphery (hematoxylin and eosin stain X600).