BACKGROUND: Necrobiotic xanthogranuloma (NXG) is a rare chronic disorder characterized by firm yellow to red-orange nodules and plaques affecting the face, abdomen, and extremities with the potential for systemic involvement. NXG has a close association with monoclonal gammopathies, and there is a predilection for the development of multiple myeloma. Treatment options are varied due to inconsistent results seen with the use of corticosteroids, immunomodulators, chemotherapeutic agents, and antibiotics. OBJECTIVE: We describe a patient with smoldering multiple myeloma associated with progressive NXG successfully treated with high-dose intravenous immunoglobulin (IVIG). CONCLUSION: Our case adds to the single previous report of two cases of NXG with significant improvement from treatment with IVIG and confirms the efficacy of this treatment modality.
BACKGROUND:Necrobiotic xanthogranuloma (NXG) is a rare chronic disorder characterized by firm yellow to red-orange nodules and plaques affecting the face, abdomen, and extremities with the potential for systemic involvement. NXG has a close association with monoclonal gammopathies, and there is a predilection for the development of multiple myeloma. Treatment options are varied due to inconsistent results seen with the use of corticosteroids, immunomodulators, chemotherapeutic agents, and antibiotics. OBJECTIVE: We describe a patient with smoldering multiple myeloma associated with progressive NXG successfully treated with high-dose intravenous immunoglobulin (IVIG). CONCLUSION: Our case adds to the single previous report of two cases of NXG with significant improvement from treatment with IVIG and confirms the efficacy of this treatment modality.
Authors: Caroline A Nelson; Connie S Zhong; David A Hashemi; Hovik J Ashchyan; Zoe Brown-Joel; Megan H Noe; Sotonye Imadojemu; Robert G Micheletti; Ruth Ann Vleugels; Karolyn A Wanat; Misha Rosenbach; Arash Mostaghimi Journal: JAMA Dermatol Date: 2020-03-01 Impact factor: 10.282
Authors: Lisa Steinhelfer; Thomas Kühnel; Herbert Jägle; Stephanie Mayer; Sigrid Karrer; Frank Haubner; Stephan Schreml Journal: Orphanet J Rare Dis Date: 2022-03-24 Impact factor: 4.123