An official website of the United States government
The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before
sharing sensitive information, make sure you’re on a federal
government site.
The site is secure.
The https:// ensures that you are connecting to the
official website and that any information you provide is encrypted
and transmitted securely.
Figure.. Periorbital Lesions in 3 Patients and the Hypothesized Partial Mechanism of Necrobiotic Xanthogranuloma (NXG)…
Figure.. Periorbital Lesions in 3 Patients and the Hypothesized Partial Mechanism of Necrobiotic Xanthogranuloma (NXG) Associated With ABCG5/G8 Variants
D-F, Histopathologic examination showed palisading granulomas (black arrowhead) in with lymphoplasmacytic infiltration and zones of necrobiosis (asterisk) (D), abundant peripheral foam cells (yellow arrowhead) (E), and Touton giant cells (blue arrowhead) (F) (hematoxylin-eosin; bars: 500 μm [D and E] and 250 μm [F]). G, The hypothesized partial mechanism of NXG associated with ABCG5/G8 variants involves cellular sterol accumulation and decreased high-density lipoprotein (HDL) concentrations, which lead to impaired reverse cholesterol transport (RCT), inflammatory responses, and the migration of monocytes from peripheral blood to the skin, ultimately resulting in disrupted macrophage-lipid homeostasis. This diagram was created with BioRender.com. ABCG indicates adenosine triphosphate–binding cassette subfamily G; CCR, chemokine receptor; ICAM-1, intercellular adhesion molecule 1; IL, interleukin; MCP-1, membrane cofactor protein 1; MIP-1α, macrophage inflammatory protein 1α; TNF, tumor necrosis factor; VCAM-1, vascular cellular adhesion molecule 1.
Nelson CA, Zhong CS, Hashemi DA, et al. A multicenter cross-sectional study and systematic review of necrobiotic xanthogranuloma with proposed diagnostic criteria. JAMA Dermatol. 2020;156(3):270-279. doi: 10.1001/jamadermatol.2019.4221
-
DOI
-
PMC
-
PubMed
Szalat R, Pirault J, Fermand JP, et al. Physiopathology of necrobiotic xanthogranuloma with monoclonal gammopathy. J Intern Med. 2014;276(3):269-284. doi: 10.1111/joim.12195
-
DOI
-
PMC
-
PubMed
Xia Y, Duan Y, Zheng W, et al. Clinical, genetic profile and therapy evaluation of 55 children and 5 adults with sitosterolemia. J Clin Lipidol. 2022;16(1):40-51. doi: 10.1016/j.jacl.2021.11.015
-
DOI
-
PubMed
Farhat D, Rezaei F, Ristovski M, et al. Structural analysis of cholesterol binding and sterol selectivity by ABCG5/G8. J Mol Biol. 2022;434(20):167795. doi: 10.1016/j.jmb.2022.167795
-
DOI
-
PubMed
Dikkers A, Freak de Boer J, Annema W, Groen AK, Tietge UJ. Scavenger receptor BI and ABCG5/G8 differentially impact biliary sterol secretion and reverse cholesterol transport in mice. Hepatology. 2013;58(1):293-303. doi: 10.1002/hep.26316
-
DOI
-
PubMed