Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin

Background: Necrobiotic xanthogranuloma (NXG) is a rare non-Langerhans histiocytosis with cutaneous manifestations, most commonly of the periorbital skin, and is often associated with hematologic disorders such as monoclonal gammopathy. Treatment of NXG is notoriously difficult, and fraught with rec...

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Main Authors: Rose M. Olson, Andrew R. Harrison, Amanda Maltry, Ali Mokhtarzadeh
Format: Article
Language:English
Published: Karger Publishers 2018-01-01
Series:Case Reports in Ophthalmology
Subjects:
Online Access:https://www.karger.com/Article/FullText/485913
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author Rose M. Olson
Andrew R. Harrison
Amanda Maltry
Ali Mokhtarzadeh
author_facet Rose M. Olson
Andrew R. Harrison
Amanda Maltry
Ali Mokhtarzadeh
author_sort Rose M. Olson
collection DOAJ
description Background: Necrobiotic xanthogranuloma (NXG) is a rare non-Langerhans histiocytosis with cutaneous manifestations, most commonly of the periorbital skin, and is often associated with hematologic disorders such as monoclonal gammopathy. Treatment of NXG is notoriously difficult, and fraught with recurrence and progression. Case Presentation: The authors describe a case of NXG with periorbital involvement in a patient with a complex autoimmune and hematologic medical history. The biopsy of this rare lesion prompted subsequent evaluation for an underlying disorder, which led to the diagnosis of multiple myeloma. Her NXG lesions demonstrated remarkable clinical improvement after treatment with intravenous immunoglobulin (IVIG). Conclusions: This case demonstrates the ophthalmologist’s critical role in the diagnosis and management of NXG, as early detection cannot only prevent ophthalmic consequences such as ocular perforation and blindness, but also prompt further investigation that may reveal an underlying disorder or systemic involvement, including hematologic malignancy as in this case. NXG has been effectively treated with IVIG in a handful of reported cases. To the author’s knowledge, this is the third case of periorbital NXG successfully treated with IVIG, and the first in the ophthalmic literature.
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spelling doaj.art-cb12132b35204a059c5ecb5a1726c2422022-12-21T19:19:24ZengKarger PublishersCase Reports in Ophthalmology1663-26992018-01-0191768110.1159/000485913485913Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous ImmunoglobulinRose M. OlsonAndrew R. HarrisonAmanda MaltryAli MokhtarzadehBackground: Necrobiotic xanthogranuloma (NXG) is a rare non-Langerhans histiocytosis with cutaneous manifestations, most commonly of the periorbital skin, and is often associated with hematologic disorders such as monoclonal gammopathy. Treatment of NXG is notoriously difficult, and fraught with recurrence and progression. Case Presentation: The authors describe a case of NXG with periorbital involvement in a patient with a complex autoimmune and hematologic medical history. The biopsy of this rare lesion prompted subsequent evaluation for an underlying disorder, which led to the diagnosis of multiple myeloma. Her NXG lesions demonstrated remarkable clinical improvement after treatment with intravenous immunoglobulin (IVIG). Conclusions: This case demonstrates the ophthalmologist’s critical role in the diagnosis and management of NXG, as early detection cannot only prevent ophthalmic consequences such as ocular perforation and blindness, but also prompt further investigation that may reveal an underlying disorder or systemic involvement, including hematologic malignancy as in this case. NXG has been effectively treated with IVIG in a handful of reported cases. To the author’s knowledge, this is the third case of periorbital NXG successfully treated with IVIG, and the first in the ophthalmic literature.https://www.karger.com/Article/FullText/485913Necrobiotic xanthogranulomaNon-Langerhans histiocytosisIntravenous immunoglobulin
spellingShingle Rose M. Olson
Andrew R. Harrison
Amanda Maltry
Ali Mokhtarzadeh
Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin
Case Reports in Ophthalmology
Necrobiotic xanthogranuloma
Non-Langerhans histiocytosis
Intravenous immunoglobulin
title Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin
title_full Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin
title_fullStr Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin
title_full_unstemmed Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin
title_short Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin
title_sort periorbital necrobiotic xanthogranuloma successfully treated with intravenous immunoglobulin
topic Necrobiotic xanthogranuloma
Non-Langerhans histiocytosis
Intravenous immunoglobulin
url https://www.karger.com/Article/FullText/485913
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AT amandamaltry periorbitalnecrobioticxanthogranulomasuccessfullytreatedwithintravenousimmunoglobulin
AT alimokhtarzadeh periorbitalnecrobioticxanthogranulomasuccessfullytreatedwithintravenousimmunoglobulin