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Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin.
Case Rep Ophthalmol 2018 Jan-Apr; 9(1):70-75CR

Abstract

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.

Authors+Show Affiliations

University of Minnesota School of Medicine, Minneapolis, Minnesota, USA.Department of Ophthalmology and Visual Neurosciences, University of Minnesota, Minneapolis, Minnesota, USA. Department of Ophthalmology and Otolaryngology, University of Minnesota, Minneapolis, Minnesota, USA.Department of Ophthalmology and Visual Neurosciences, University of Minnesota, Minneapolis, Minnesota, USA.Department of Ophthalmology and Visual Neurosciences, University of Minnesota, Minneapolis, Minnesota, USA.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

29643785

Citation

Olson, Rose M., et al. "Periorbital Necrobiotic Xanthogranuloma Successfully Treated With Intravenous Immunoglobulin." Case Reports in Ophthalmology, vol. 9, no. 1, 2018, pp. 70-75.
Olson RM, Harrison AR, Maltry A, et al. Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin. Case Rep Ophthalmol. 2018;9(1):70-75.
Olson, R. M., Harrison, A. R., Maltry, A., & Mokhtarzadeh, A. (2018). Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin. Case Reports in Ophthalmology, 9(1), pp. 70-75. doi:10.1159/000485913.
Olson RM, et al. Periorbital Necrobiotic Xanthogranuloma Successfully Treated With Intravenous Immunoglobulin. Case Rep Ophthalmol. 2018;9(1):70-75. PubMed PMID: 29643785.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin. AU - Olson,Rose M, AU - Harrison,Andrew R, AU - Maltry,Amanda, AU - Mokhtarzadeh,Ali, Y1 - 2018/01/17/ PY - 2017/08/14/received PY - 2017/11/29/accepted PY - 2018/4/13/entrez PY - 2018/4/13/pubmed PY - 2018/4/13/medline KW - Intravenous immunoglobulin KW - Necrobiotic xanthogranuloma KW - Non-Langerhans histiocytosis SP - 70 EP - 75 JF - Case reports in ophthalmology JO - Case Rep Ophthalmol VL - 9 IS - 1 N2 - 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. SN - 1663-2699 UR - https://www.unboundmedicine.com/medline/citation/29643785/Periorbital_Necrobiotic_Xanthogranuloma_Successfully_Treated_with_Intravenous_Immunoglobulin_ L2 - https://www.karger.com?DOI=10.1159/000485913 DB - PRIME DP - Unbound Medicine ER -