Adult-onset asthma and periocular xanthogranuloma – A rare infiltrative disease of the orbit and eyelid
Purpose: To present a case of adult onset asthma with periocular xanthogranuloma (AAPOX), and discuss existing literature on adult orbital xanthogranulomatous diseases (AOXGDs) and their treatment. Observations: A 63 year old male presented with progressive bilateral eyelid swelling with overlying y...
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| Format: | Article |
| Language: | English |
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Elsevier
2021-06-01
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| Series: | American Journal of Ophthalmology Case Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2451993621000347 |
| _version_ | 1819101187253731328 |
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| author | Michael B. Green Mary K. Daly Nora M.V. Laver Daniel R. Lefebvre |
| author_facet | Michael B. Green Mary K. Daly Nora M.V. Laver Daniel R. Lefebvre |
| author_sort | Michael B. Green |
| collection | DOAJ |
| description | Purpose: To present a case of adult onset asthma with periocular xanthogranuloma (AAPOX), and discuss existing literature on adult orbital xanthogranulomatous diseases (AOXGDs) and their treatment. Observations: A 63 year old male presented with progressive bilateral eyelid swelling with overlying yellow plaques associated with asthma. CT scan showed periorbital swelling with enlargement of the superior and lateral rectus muscles bilaterally. Biopsy demonstrated orbital xanthogranulomatous disease with increased IgG4 plasma cells. The patient was treated with intralesional triamcinolone, oral prednisone, and cyclophosphamide without significant improvement. Surgical debulking was eventually performed which improved his external symptoms until he was lost to follow up 15 months later. Conclusions and Importance: AOXGDs are a group of rare infiltrative diseases of the eyelids and orbit that can be associated with significant systemic morbidities. While they all have similar underlying histopathologic features, appreciating the clinical difference between these diseases is important in understanding patient prognosis and ensuring appropriate clinical monitoring. There is also growing research demonstrating that AAPOX, along with other AOXGDs, may represent part of a continuum of IgG4 related disease, similar to what is seen in this case. There is currently no reliably effective treatment for AOXGDs, and additional research into the management of these diseases is necessary. |
| first_indexed | 2024-12-22T01:14:41Z |
| format | Article |
| id | doaj.art-903b3d4bb8f04065a00157a6a017e1ff |
| institution | Directory Open Access Journal |
| issn | 2451-9936 |
| language | English |
| last_indexed | 2024-12-22T01:14:41Z |
| publishDate | 2021-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | American Journal of Ophthalmology Case Reports |
| spelling | doaj.art-903b3d4bb8f04065a00157a6a017e1ff2022-12-21T18:43:54ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362021-06-0122101043Adult-onset asthma and periocular xanthogranuloma – A rare infiltrative disease of the orbit and eyelidMichael B. Green0Mary K. Daly1Nora M.V. Laver2Daniel R. Lefebvre3Veterans Affairs Boston Healthcare System, Department of Ophthalmology, 150 South Huntington Avenue, Boston, MA, 02130, USA; Boston University School of Medicine, Department of Ophthalmology, 72 East Concord Street, Boston, MA, 02118, USAVeterans Affairs Boston Healthcare System, Department of Ophthalmology, 150 South Huntington Avenue, Boston, MA, 02130, USA; Boston University School of Medicine, Department of Ophthalmology, 72 East Concord Street, Boston, MA, 02118, USA; Harvard Medical School, Department of Ophthalmology, 243 Charles Street, Boston, MA, 02114, USATufts University School of Medicine, Department of Ophthalmology and Department of Pathology and Laboratory Medicine, 800 Washington Street, Boston, MA, 02111, USAVeterans Affairs Boston Healthcare System, Department of Ophthalmology, 150 South Huntington Avenue, Boston, MA, 02130, USA; Harvard Medical School, Department of Ophthalmology, 243 Charles Street, Boston, MA, 02114, USA; Corresponding author. Veterans Affairs Boston Healthcare System, Department of Ophthalmology, 150 South Huntington Ave. Boston, MA, 02130, USA.Purpose: To present a case of adult onset asthma with periocular xanthogranuloma (AAPOX), and discuss existing literature on adult orbital xanthogranulomatous diseases (AOXGDs) and their treatment. Observations: A 63 year old male presented with progressive bilateral eyelid swelling with overlying yellow plaques associated with asthma. CT scan showed periorbital swelling with enlargement of the superior and lateral rectus muscles bilaterally. Biopsy demonstrated orbital xanthogranulomatous disease with increased IgG4 plasma cells. The patient was treated with intralesional triamcinolone, oral prednisone, and cyclophosphamide without significant improvement. Surgical debulking was eventually performed which improved his external symptoms until he was lost to follow up 15 months later. Conclusions and Importance: AOXGDs are a group of rare infiltrative diseases of the eyelids and orbit that can be associated with significant systemic morbidities. While they all have similar underlying histopathologic features, appreciating the clinical difference between these diseases is important in understanding patient prognosis and ensuring appropriate clinical monitoring. There is also growing research demonstrating that AAPOX, along with other AOXGDs, may represent part of a continuum of IgG4 related disease, similar to what is seen in this case. There is currently no reliably effective treatment for AOXGDs, and additional research into the management of these diseases is necessary.http://www.sciencedirect.com/science/article/pii/S2451993621000347Adult orbital xanthogranulomatous diseaseXanthogranulomaAdult onset asthma with periocular xanthogranulomaIgG4-related diseaseNon-langerhans cell histiocytosisInfiltrative diseases of the orbit and eyelid |
| spellingShingle | Michael B. Green Mary K. Daly Nora M.V. Laver Daniel R. Lefebvre Adult-onset asthma and periocular xanthogranuloma – A rare infiltrative disease of the orbit and eyelid American Journal of Ophthalmology Case Reports Adult orbital xanthogranulomatous disease Xanthogranuloma Adult onset asthma with periocular xanthogranuloma IgG4-related disease Non-langerhans cell histiocytosis Infiltrative diseases of the orbit and eyelid |
| title | Adult-onset asthma and periocular xanthogranuloma – A rare infiltrative disease of the orbit and eyelid |
| title_full | Adult-onset asthma and periocular xanthogranuloma – A rare infiltrative disease of the orbit and eyelid |
| title_fullStr | Adult-onset asthma and periocular xanthogranuloma – A rare infiltrative disease of the orbit and eyelid |
| title_full_unstemmed | Adult-onset asthma and periocular xanthogranuloma – A rare infiltrative disease of the orbit and eyelid |
| title_short | Adult-onset asthma and periocular xanthogranuloma – A rare infiltrative disease of the orbit and eyelid |
| title_sort | adult onset asthma and periocular xanthogranuloma a rare infiltrative disease of the orbit and eyelid |
| topic | Adult orbital xanthogranulomatous disease Xanthogranuloma Adult onset asthma with periocular xanthogranuloma IgG4-related disease Non-langerhans cell histiocytosis Infiltrative diseases of the orbit and eyelid |
| url | http://www.sciencedirect.com/science/article/pii/S2451993621000347 |
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