By Gary M. White, MD
Courtesy Theodore Sebastian, MD
Necrobiotic xanthogranuloma (NX) is a rare condition in which xanthogranulomas develop usually about the eyes, in association with a paraprotein.
Periorbital yellow nodules and plaques which may ulcerate occur in necrobiotic xanthogranuloma. Lesions may be violaceous or xanthomatous and may occur anywhere on the extremities or trunk. Ulceration is common. Keratitis, scleritis, and anterior uveitis may occur as well as hepatosplenomegaly. Bone marrow plasmacytosis is common but multiple myeloma is rare. B-cell lymphoma has been associated. Complement abnormalities have been associated--presumably related to the paraprotein.
A woman with dramatic hyperpigmentation of the face caused by NXG has been reported [JAMA Derm 2014;150;1221 (CP)].
Initial workup may include SPEP, UPEP, CBC, C4, C3, CH50, lipid profile, cryoglobulins, ANA, and ophthalmologic exam. Routine followup should include a CBC to exclude myeloma.
Referral to a hematologist/oncologist is in order. Low dose chlorambucil (e.g., 2-4 mg/d) and melphalan have been used with near complete resolution in some patients. Other therapies include steroids (topical IL and/or systemic), recombinant IFN alfa-2a, phototherapy, thalidomide, lenalidomide, IV immunoglobulin and plasmapheresis. The cutaneous lesions of one patient responded to topical nitrogen mustard (mechlorethamine 0.01% ointment nightly) [JAMADerm 2016;152;589].
A recent review of reported treatments may be found here [JEADV 2017;31;221].
Long-term followup for the development of lymphoproliferative disease, e.g. multiple myeloma is necessary.
Indian J Dermatol 2012;57:396-8
Journal of Cut and Aesth Surgery
Dermatology Online Journal 12(1)
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