Nelson et al reported criteria for the diagnosis of Necrobiotic Xanthogranuloma. The authors are from Brigham and Women's Hospital, University of Pennsylvania, and the University of Iowa.

Major criteria:

(1) cutaneous lesions (papules, plaques, nodules) usually yellow or orange in color

(2) histology shows palisading granulomas with zones of necrobiosis


Minor criteria:

(1) paraproteinemia, plasma cell dyscrasia or other lymphoproliferative disorder

(2) periorbital distribution of skin lesions



• The paraproteinemia is often IgG-kappa.

• Extracutaneous involvement may be seen in eye, liver, GI tract, or other sites.


The diagnosis is made with:

(1) both major criteria

(2) >= 1 minor criteria

(3) exclusion of other cause for granuloma (foreign body, infection, etc).

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