Sclerosing Wegener's granulomatosis in the orbit.
OBJECTIVE
To report three cases of sclerosing Wegener's granulomatosis in the orbit and to compare the histopathological morphology with those of other types of sclerosing orbital inflammation.


METHODS
We analysed the clinical data along with histopathological specimens from orbital biopsies and from two enucleated eyes.


RESULTS
All three patients had longstanding inflammation in the orbit and involvement of the paranasal sinuses. Proptosis, impaired ocular motility and reduced visual acuity dominated the clinical picture. All histopathological specimens featured granulomatous inflammation, tissue necrosis, vasculitis and widespread dense fibrosis. In certain areas of all specimens the fibrous tissue was arranged in characteristic concentric, onionskin-like whorls of collagen around obliterated small blood vessels.


CONCLUSIONS
Wegener's granulomatosis may lead to orbital fibrosis and sclerosis. The stromal changes and cellular infiltrate resemble the non-specific orbital inflammation previously termed orbital pseudotumour. We present the first 'onionskin' lesions in association with Wegener's granulomatosis in the orbit. This latter morphology may represent an abnormal fibrotic reaction in inflammatory tissue.
Department of Neuroscience and Pharmacology, Eye Pathology Section, University of Copenhagen, Copenhagen, Denmark.
Christoffer
C
Ostri
Steffen
S
Heegaard
Jan Ulrik
JU
Prause
D005094:Exophthalmos / Q000209:etiology; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D009916:Orbital Diseases / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D010256:Paranasal Sinuses / Q000473:pathology; D012598:Sclerosis; D014657:Vasculitis / Q000209:etiology; D014792:Visual Acuity