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
where:
• 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).
