Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies

Adult onset xanthogranuloma – case report and review of literature

“…An aberrant response of histiocytes to yet unknown stimuli, most probably physical or infectious is suspected to be the underlying mechanism of the disease, the ensuing foreign body-type reaction leading to the appearance of cutaneous and/or extracutaneous lesions (16,17). Other proposed triggers for the granulomatous reaction are complexes of serum immunoglobulins and lipids or the binding of monoclonal proteins to lipoprotein receptors on monocytes (18,19). JXG has been described in association with a series of diseases, such as neurofibromatosis type 1 (NF1), Niemann-Pick disease, juvenile myelomonocytic leukemia (JMML), and urticaria pigmentosa (20)(21)(22).…”

Necrobiotic xanthogranuloma associated with chronic lymphocytic leukemia

“…2 Up to 80% NXG are associated with IgGk-monoclonal gammopathy, which can be detected by the presence of M protein in the serum protein electrophoresis. 2,3,5 The exact pathogenesis of NXG still remains unclear. 2,3 Besides these most common plasmaproliferative diseases, other hematolymphoid malignancy such as lympho-and myeloproliferative diseases have been described with NXG as an indicator for the underlying malignancy.…”

“…[2][3][4] In the literature, only a few additional cases reporting the association between NXG and chronic lymphocytic leukemia (CLL) can be found. 2,3,5 The exact pathogenesis of NXG still remains unclear. Several potential pathological mechanisms are discussed including the binding of paraproteins to histiocytes receptors, imitating the function of a lipoprotein, resulting in xanthogranuloma formation.…”

Extrafacial necrobiotic xanthogranuloma and monoclonal gammopathy: two cases

“…Other sites such as the trunk, limbs, and scalp may also be affected and are very frequently associated with facial lesions. Far less frequently, lesions occur without any concomitant facial lesions …”