Bilateral epibulbar pseudorheumatoid nodulosis with a review of ocular adnexal palisading granulomas

Marisa G Tieger; Frederick A Jakobiec; Natalie Wolkow; Lina Ma; H Peggy Chang

doi:10.1016/j.survophthal.2019.02.002

Bilateral epibulbar pseudorheumatoid nodulosis with a review of ocular adnexal palisading granulomas

Surv Ophthalmol. 2019 Jul-Aug;64(4):558-569. doi: 10.1016/j.survophthal.2019.02.002. Epub 2019 Feb 14.

Authors

Marisa G Tieger¹, Frederick A Jakobiec², Natalie Wolkow¹, Lina Ma¹, H Peggy Chang³

Affiliations

¹ David G Cogan Laboratory of Ophthalmic Pathology, Boston, MA, USA; Massachusetts Eye and Ear Infirmary and the Harvard Medical School, Boston, MA, USA.
² David G Cogan Laboratory of Ophthalmic Pathology, Boston, MA, USA; Massachusetts Eye and Ear Infirmary and the Harvard Medical School, Boston, MA, USA. Electronic address: Fred_Jakobiec@meei.harvard.edu.
³ Massachusetts Eye and Ear Infirmary and the Harvard Medical School, Boston, MA, USA; Cornea and Refractive Surgery Service, Boston, MA, USA.

PMID: 30772365
DOI: 10.1016/j.survophthal.2019.02.002

Abstract

We describe the clinical, histopathologic, and immunohistochemical characteristics of episcleral/conjunctival pseudorheumatoid nodulosis, a new granulomatous entity that belongs among a group of related lesions. Specifically, pseudorheumatoid nodulosis should be differentiated from solitary rheumatoid nodules, rheumatoid nodulosis, accelerated rheumatoid nodules and nodulosis, and solitary pseudorheumatoid nodules. A 53-year-old man presented with bilateral painless, large, faintly yellow-gray, partially immobile, solid, circumscribed, and occasionally confluent episcleral nodules of several months' duration. He had never had clinical rheumatoid arthritis and was rheumatoid factor negative. Biopsy revealed multiple, merging episcleral/conjunctival, nonulcerated, palisading granulomas with variably sized central zones of necrobiosis of collagen. Abundant palisading CD68/163 + histiocytes admixed with fibroblasts surrounded the necrobiotic foci, which failed to stain with Alcian blue for mucopolysaccharides. No fibrinoid deposits were detected. Numerous CD3+ T lymphocytes, fewer CD 20 + B lymphocytes, and a smaller subpopulation of CD138 + plasma cells were present. Numerous CD1a + Langerhans cells were scattered among the palisading histiocytes and overlying epithelium. Immunohistochemical stains for immunoglobulins revealed concentrations of IgG, IgM, and IgA, but not IgE, in the necrobiotic zones. Special stains did not reveal evidence of infection nor did polarization microscopy display any foreign material. An extensive systemic and serologic workup was negative. We review simulating palisading or other nonrheumatic granulomas that should be distinguished from pseudorheumatoid nodules or nodulosis and explore therapeutic options.

Keywords: accelerated rheumatoid nodulosis; conjunctiva; episclera; palisading granuloma; pseudorheumatoid nodule; pseudorheumatoid nodulosis; rheumatoid arthritis; rheumatoid nodule; rheumatoid nodulosis.

Publication types

Case Reports
Research Support, Non-U.S. Gov't
Review

MeSH terms

Conjunctival Diseases* / immunology
Conjunctival Diseases* / pathology
Granuloma* / immunology
Granuloma* / pathology
Humans
Male
Middle Aged
Rheumatoid Nodule* / immunology
Rheumatoid Nodule* / pathology