Progressive sclerosing orbital pseudotumors are a subset of usually primary and localized idiopathic fibroinflammatory disorders. We report on a 66-year-old man who developed this condition along the facial tissue planes with extension into the orbit and preferential involvement of the periorbital membrane. Fibrocollagenous tissue with scattered lymphoid aggregates without follicle formation dominated the process. There was a light dispersion of B and T lymphocytes and histiocytes in the stroma. Immunoglobulin G4 (IgG4)-positive plasma cells (35 per high-power field) were identified mostly in the lymphoid clusters, as has been discovered in similar IgG4-related fibrosclerosing conditions of other nonorbital sites. No associated systemic disease emerged during a 20-year clinical course. Previously reported orbital cases of IgG4-positive disease have all involved the lacrimal gland, usually bilaterally, and more closely resembled hypercellular reactive lymphoid hyperplasias with moderate interlobular fibrosis, rather than representing an essentially sclerosing process from the beginning.
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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