Idiopathic Fibroinflammatory Disease of the Face, Eyelids, and Periorbital Membrane With Immunoglobu - PDF by ProQuest


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									        Idiopathic Fibroinflammatory Disease of the Face,
             Eyelids, and Periorbital Membrane With
            Immunoglobulin G4–Positive Plasma Cells
                                  Manisha Mehta, MD; Frederick Jakobiec, MD, DSc; Aaron Fay, MD

● Progressive sclerosing orbital pseudotumors are a subset               also be accompanying polymorphonuclear and eosino-
of usually primary and localized idiopathic fibroinflam-                   philic leukocytes, particularly in childhood cases.3 Gran-
matory disorders. We report on a 66-year-old man who                     ulomatous inflammation and angiodestructive inflamma-
developed this condition along the facial tissue planes with             tion are generally absent. Clinically and pathologically, the
extension into the orbit and preferential involvement of the             disease must be distinguished from Wegener granuloma-
periorbital membrane. Fibrocollagenous tissue with scat-                 tosis, its closest simulant.4 In the past, the spectrum of
tered lymphoid aggregates without follicle formation dom-                pseudotumors has often been subsumed with ocular ad-
inated the process. There was a light dispersion of B and T              nexal lymphoid neoplasms, both hypercellular reactive
lymphocytes and histiocytes in the stroma. Immunoglobu-                  follicular hyperplasias and small cell monoclonal B-cell
lin G4 (IgG4)–positive plasma cells ( 35 per high-power                  proliferations usually devoid of a prominent collagenous
field) were identified mostly in the lymphoid clusters, as                 stroma.1,2
has been discovered in similar IgG4–related fibrosclerosing                  Most fibroinflammatory lesions are localized to the or-
conditions of other nonorbital sites. No associated system-              bit, but multifocal systemic disease with involvement of
ic disease emerged during a 20-year clinical course. Pre-                the mediastinum and/or retroperitoneum along with the
viously reported orbital cases of IgG4-positive disease have             orbit is well known to occur.1 A relationship of orbital le-
all involved the lacrimal gland, usually bilaterally, and                sions has been suggested recently to a new entity of im-
more closely resembled hypercellular reactive lymphoid                   munoglobulin G4 (IgG4)–related inflammatory diseases.
hyperplasias with moderate interlobular fibrosis, rather                  Autoimmune sclerosing pancreatitis, retroperitoneal fibro-
than representing an essentially sclerosing process from the             sis, and sclerosing cholangitis are examples of preferential
beginning.                                                               sites, although other loci of disease embrace the kidney,
   (Arch Pathol Lab Med. 2009;133:1251–1255)                             lung, prostate, and even the hypophysis.5 We report a var-
                                                                         iant of orbital fibroinflammatory disease with prominent
                                                                         IgG4 plasma cells in the biopsied tissues that had an un-
T  he sclerosing form of idiopathic orbital inflammation
     (also called pseudotumor and probably better desig-
nated as fibroinflammatory disease) constitutes a small sub-
                                                                         usual clinical presentation; we do not believe that the pres-
                                                                         ent constellation of findings has been described previously
                                                                         in the literature. It entailed a massive facial swelling with
set of noninfectious inflammatory diseases, which overall                 an interconnected and contiguous sclerosing orbital pro-
comprise roughly 5% to 8% of orbital biopsies.1–3 It is an               cess. Retention of excellent visual acuity and extraocular
aggressive, quasineoplastic (tumefactive) subtype that                   motility was paradoxically permitted because the orbital
poses many diagnostic and therapeutic challenges. It is                  component mostly centered on involvement of the peri-
characterized by an intractably spreading and progres-                   orbital fibrous membrane (periosteum) covering the inner
sively collagenizing stroma that infiltrates the orbital                  aspect of the orbital bones.
structures and contains a dispersion of lymphocytes,
which m
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