{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T15:09:49Z","timestamp":1770822589497,"version":"3.50.1"},"reference-count":30,"publisher":"Archives of Pathology and Laboratory Medicine","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2009,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>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)\u2013positive plasma cells (&gt;35 per high-power field) were identified mostly in the lymphoid clusters, as has been discovered in similar IgG4\u2013related 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.<\/jats:p>","DOI":"10.5858\/133.8.1251","type":"journal-article","created":{"date-parts":[[2021,2,12]],"date-time":"2021-02-12T10:42:10Z","timestamp":1613126530000},"page":"1251-1255","source":"Crossref","is-referenced-by-count":62,"title":["Idiopathic Fibroinflammatory Disease of the Face, Eyelids, and Periorbital Membrane With Immunoglobulin G4\u2013Positive Plasma Cells"],"prefix":"10.5858","volume":"133","author":[{"given":"Manisha","family":"Mehta","sequence":"first","affiliation":[{"name":"From the Department of Ophthalmology (Drs Mehta, Jakobiec, and Fay), the David G. Cogan Laboratory of Ophthalmic Pathology (Drs Jakobiec and Mehta), and the Facial Plastic and Reconstructive Surgery Service (Dr Fay), Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frederick","family":"Jakobiec","sequence":"additional","affiliation":[{"name":"From the Department of Ophthalmology (Drs Mehta, Jakobiec, and Fay), the David G. Cogan Laboratory of Ophthalmic Pathology (Drs Jakobiec and Mehta), and the Facial Plastic and Reconstructive Surgery Service (Dr Fay), Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aaron","family":"Fay","sequence":"additional","affiliation":[{"name":"From the Department of Ophthalmology (Drs Mehta, Jakobiec, and Fay), the David G. Cogan Laboratory of Ophthalmic Pathology (Drs Jakobiec and Mehta), and the Facial Plastic and Reconstructive Surgery Service (Dr Fay), Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"3906","reference":[{"key":"i1543-2165-133-8-1251-b1","article-title":"Orbit.","author":"Jakobiec, F. A.","unstructured":"Jakobiec, F. A. and R. L.Font. Orbit.In: Spencer WH, ed. Ophthalmic Pathology: An Atlas and Textbook. 4th ed. Philadelphia, PA: WB Saunders; 1996:2822\u20132844."},{"issue":"6","key":"i1543-2165-133-8-1251-b2","series-title":"Am J Ophthalmol","first-page":"941","article-title":"Ocular adnexal lymphoid tumors: progress in need of clarification.","volume":"145","author":"Jakobiec, F. A.","year":"2008","unstructured":"Jakobiec, F. A.\n          \n          Ocular adnexal lymphoid tumors: progress in need of clarification.\n          Am J Ophthalmol\n          2008. 1456:941\u2013950."},{"issue":"6","key":"i1543-2165-133-8-1251-b3","series-title":"Ophthalmology","first-page":"565","article-title":"Idiopathic inflammatory orbital pseudotumor in childhood: II: results of diagnostic tests and biopsies.","volume":"88","author":"Mottow-Lippa, L.","year":"1981","unstructured":"Mottow-Lippa, L., F. A.Jakobiec, and M.Smith. Idiopathic inflammatory orbital pseudotumor in childhood: II: results of diagnostic tests and biopsies.Ophthalmology1981. 886:565\u2013574."},{"issue":"1","key":"i1543-2165-133-8-1251-b4","series-title":"Int Ophthalmol","first-page":"35","article-title":"Diagnosis of limited ophthalmic Wegener granulomatosis: distinctive pathologic features with ANCA test confirmation.","volume":"28","author":"Ahmed, M.","year":"2008","unstructured":"Ahmed, M., J. H.Niffenegger, and F. A.Jakobiec. et al. Diagnosis of limited ophthalmic Wegener granulomatosis: distinctive pathologic features with ANCA test confirmation.Int Ophthalmol2008. 281:35\u201346."},{"issue":"25","key":"i1543-2165-133-8-1251-b5","series-title":"World J Gastroenterol","first-page":"3948","article-title":"IgG4-related sclerosing disease.","volume":"14","author":"Kamisawa, T.","year":"2008","unstructured":"Kamisawa, T. and A.Okamoto. IgG4-related sclerosing disease.World J Gastroenterol2008. 1425:3948\u20133955."},{"issue":"10","key":"i1543-2165-133-8-1251-b6","series-title":"N Engl J Med","first-page":"732","article-title":"High serum IgG4 concentrations in patients with sclerosing pancreatitis.","volume":"344","author":"Hamano, H.","year":"2001","unstructured":"Hamano, H., S.Kawa, and A.Horiuchi. et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis.N Engl J Med2001. 34410:732\u2013738."},{"issue":"8","key":"i1543-2165-133-8-1251-b7","series-title":"Am J Surg Pathol","first-page":"1159","article-title":"Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease.","volume":"32","author":"Cheuk, W.","year":"2008","unstructured":"Cheuk, W., H. K.Yuen, and A. C.Chan. et al. Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease.Am J Surg Pathol2008. 328:1159\u20131167."},{"issue":"4","key":"i1543-2165-133-8-1251-b8","series-title":"Am J Surg Pathol","first-page":"643","article-title":"Chronic sclerosing dacryoadenitis: part of the spectrum of IgG4-related Sclerosing disease?","volume":"31","author":"Cheuk, W.","year":"2007","unstructured":"Cheuk, W., H. K.Yuen, and J. K.Chan. Chronic sclerosing dacryoadenitis: part of the spectrum of IgG4-related Sclerosing disease?Am J Surg Pathol2007. 314:643\u2013645."},{"issue":"8","key":"i1543-2165-133-8-1251-b9","series-title":"Pathol Int","first-page":"465","article-title":"Ocular adnexal IgG4-related disease has uniform clinicopathology.","volume":"58","author":"Sato, Y.","year":"2008","unstructured":"Sato, Y., K.Ohshima, and K.Ichimura. et al. Ocular adnexal IgG4-related disease has uniform clinicopathology.Pathol Int2008. 588:465\u2013470."},{"issue":"11","key":"i1543-2165-133-8-1251-b10","series-title":"Arch Ophthalmol","first-page":"1575","article-title":"IgG4-related chronic sclerosing dacryoadenitis.","volume":"125","author":"Takahira, M.","year":"2007","unstructured":"Takahira, M., M.Kawano, and Y.Zen. et al. IgG4-related chronic sclerosing dacryoadenitis.Arch Ophthalmol2007. 12511:1575\u20131578."},{"issue":"1","key":"i1543-2165-133-8-1251-b11","series-title":"Mod Pathol","first-page":"23","article-title":"IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis.","volume":"20","author":"Zhang, L.","year":"2007","unstructured":"Zhang, L., K.Notohara, and M. J.Levy. et al. IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis.Mod Pathol2007. 201:23\u201328."},{"issue":"1","key":"i1543-2165-133-8-1251-b12","series-title":"Ophthalmology","first-page":"100","article-title":"The immunoarchitecture of the normal human lacrimal gland: relevancy for understanding pathologic conditions.","volume":"95","author":"Wieczorek, R.","year":"1988","unstructured":"Wieczorek, R., F. A.Jakobiec, E. H.Sacks, and D. M.Knowles. The immunoarchitecture of the normal human lacrimal gland: relevancy for understanding pathologic conditions.Ophthalmology1988. 951:100\u2013109."},{"key":"i1543-2165-133-8-1251-b13","series-title":"BMC Med","first-page":"23","article-title":"Hyper-IgG4 disease: report and characterisation of a new disease.","volume":"4","author":"Neild, G. H.","year":"2006","unstructured":"Neild, G. H., M.Rodriguez-Justo, C.Wall, and J. O.Connolly. Hyper-IgG4 disease: report and characterisation of a new disease.BMC Med2006. 4:23."},{"key":"i1543-2165-133-8-1251-b14","series-title":"Pancreatology","first-page":"234","article-title":"Treating patients with autoimmune pancreatitis: results from a long-term follow-up study.","volume":"5","author":"Kamisawa, T.","year":"2005","unstructured":"Kamisawa, T., M.Yoshiike, and N.Egawa. et al. Treating patients with autoimmune pancreatitis: results from a long-term follow-up study.Pancreatology2005. 5:(2\u20133). 234\u2013238. ; discussion 238\u2013240."},{"issue":"10","key":"i1543-2165-133-8-1251-b15","series-title":"J Gastroenterol","first-page":"982","article-title":"A new clinicopathological entity of IgG4-related autoimmune disease.","volume":"38","author":"Kamisawa, T.","year":"2003","unstructured":"Kamisawa, T., N.Funata, and Y.Hayashi. et al. A new clinicopathological entity of IgG4-related autoimmune disease.J Gastroenterol2003. 3810:982\u2013984."},{"key":"i1543-2165-133-8-1251-b1","article-title":"Orbit.","author":"Jakobiec, F. A.","unstructured":"Jakobiec, F. A. and R. L.Font. Orbit.In: Spencer WH, ed. Ophthalmic Pathology: An Atlas and Textbook. 4th ed. Philadelphia, PA: WB Saunders; 1996:2822\u20132844."},{"issue":"6","key":"i1543-2165-133-8-1251-b2","series-title":"Am J Ophthalmol","first-page":"941","article-title":"Ocular adnexal lymphoid tumors: progress in need of clarification.","volume":"145","author":"Jakobiec, F. A.","year":"2008","unstructured":"Jakobiec, F. A.\n          \n          Ocular adnexal lymphoid tumors: progress in need of clarification.\n          Am J Ophthalmol\n          2008. 1456:941\u2013950."},{"issue":"6","key":"i1543-2165-133-8-1251-b3","series-title":"Ophthalmology","first-page":"565","article-title":"Idiopathic inflammatory orbital pseudotumor in childhood: II: results of diagnostic tests and biopsies.","volume":"88","author":"Mottow-Lippa, L.","year":"1981","unstructured":"Mottow-Lippa, L., F. A.Jakobiec, and M.Smith. Idiopathic inflammatory orbital pseudotumor in childhood: II: results of diagnostic tests and biopsies.Ophthalmology1981. 886:565\u2013574."},{"issue":"1","key":"i1543-2165-133-8-1251-b4","series-title":"Int Ophthalmol","first-page":"35","article-title":"Diagnosis of limited ophthalmic Wegener granulomatosis: distinctive pathologic features with ANCA test confirmation.","volume":"28","author":"Ahmed, M.","year":"2008","unstructured":"Ahmed, M., J. H.Niffenegger, and F. A.Jakobiec. et al. Diagnosis of limited ophthalmic Wegener granulomatosis: distinctive pathologic features with ANCA test confirmation.Int Ophthalmol2008. 281:35\u201346."},{"issue":"25","key":"i1543-2165-133-8-1251-b5","series-title":"World J Gastroenterol","first-page":"3948","article-title":"IgG4-related sclerosing disease.","volume":"14","author":"Kamisawa, T.","year":"2008","unstructured":"Kamisawa, T. and A.Okamoto. 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Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease.Am J Surg Pathol2008. 328:1159\u20131167."},{"issue":"4","key":"i1543-2165-133-8-1251-b8","series-title":"Am J Surg Pathol","first-page":"643","article-title":"Chronic sclerosing dacryoadenitis: part of the spectrum of IgG4-related Sclerosing disease?","volume":"31","author":"Cheuk, W.","year":"2007","unstructured":"Cheuk, W., H. K.Yuen, and J. K.Chan. Chronic sclerosing dacryoadenitis: part of the spectrum of IgG4-related Sclerosing disease?Am J Surg Pathol2007. 314:643\u2013645."},{"issue":"8","key":"i1543-2165-133-8-1251-b9","series-title":"Pathol Int","first-page":"465","article-title":"Ocular adnexal IgG4-related disease has uniform clinicopathology.","volume":"58","author":"Sato, Y.","year":"2008","unstructured":"Sato, Y., K.Ohshima, and K.Ichimura. et al. Ocular adnexal IgG4-related disease has uniform clinicopathology.Pathol Int2008. 588:465\u2013470."},{"issue":"11","key":"i1543-2165-133-8-1251-b10","series-title":"Arch Ophthalmol","first-page":"1575","article-title":"IgG4-related chronic sclerosing dacryoadenitis.","volume":"125","author":"Takahira, M.","year":"2007","unstructured":"Takahira, M., M.Kawano, and Y.Zen. et al. IgG4-related chronic sclerosing dacryoadenitis.Arch Ophthalmol2007. 12511:1575\u20131578."},{"issue":"1","key":"i1543-2165-133-8-1251-b11","series-title":"Mod Pathol","first-page":"23","article-title":"IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis.","volume":"20","author":"Zhang, L.","year":"2007","unstructured":"Zhang, L., K.Notohara, and M. J.Levy. et al. IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis.Mod Pathol2007. 201:23\u201328."},{"issue":"1","key":"i1543-2165-133-8-1251-b12","series-title":"Ophthalmology","first-page":"100","article-title":"The immunoarchitecture of the normal human lacrimal gland: relevancy for understanding pathologic conditions.","volume":"95","author":"Wieczorek, R.","year":"1988","unstructured":"Wieczorek, R., F. A.Jakobiec, E. H.Sacks, and D. M.Knowles. The immunoarchitecture of the normal human lacrimal gland: relevancy for understanding pathologic conditions.Ophthalmology1988. 951:100\u2013109."},{"key":"i1543-2165-133-8-1251-b13","series-title":"BMC Med","first-page":"23","article-title":"Hyper-IgG4 disease: report and characterisation of a new disease.","volume":"4","author":"Neild, G. H.","year":"2006","unstructured":"Neild, G. H., M.Rodriguez-Justo, C.Wall, and J. O.Connolly. Hyper-IgG4 disease: report and characterisation of a new disease.BMC Med2006. 4:23."},{"key":"i1543-2165-133-8-1251-b14","series-title":"Pancreatology","first-page":"234","article-title":"Treating patients with autoimmune pancreatitis: results from a long-term follow-up study.","volume":"5","author":"Kamisawa, T.","year":"2005","unstructured":"Kamisawa, T., M.Yoshiike, and N.Egawa. et al. Treating patients with autoimmune pancreatitis: results from a long-term follow-up study.Pancreatology2005. 5:(2\u20133). 234\u2013238. ; discussion 238\u2013240."},{"issue":"10","key":"i1543-2165-133-8-1251-b15","series-title":"J Gastroenterol","first-page":"982","article-title":"A new clinicopathological entity of IgG4-related autoimmune disease.","volume":"38","author":"Kamisawa, T.","year":"2003","unstructured":"Kamisawa, T., N.Funata, and Y.Hayashi. et al. 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