Usually periorbital, but can appear elsewhere on the face, as well as extremities and trunk being common
Can ulcerate
Can be red or pink, brown, or purple
Can involve eye (most common extracutaneous manifestation), heart, GI tract, liver, and lung
Diagnostic Criteria
Proposed in 2020
Criteria are divided into major and minor
Major criteria:
Cutaneous papules, plaques, and/or nodules, most often yellow or orange in color.
Histopathological features demonstrating palisading granulomas with lymphoplasmacytic infiltrate and zones of necrobiosis. Characteristic features that are variably present include cholesterol clefts and/or giant cells (Touton or foreign body).
Minor criteria:
Paraproteinemia, most often IgG-κ, plasma-cell dyscrasia, and/or other associated lymphoproliferative disorder.
Periorbital distribution of cutaneous lesions.
Must have both major criteria plus 1 minor criterion
Management
No guidelines or well-established protocols
IVIg, antimalarials, intralesional triamcinolone, chemotherapy, lenalidomide or thalidomide have all been found to be helpful in case series
Further Reading
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
