Necrobiotic Xanthogranuloma

A cutaneous necrobiotic disorder characterized by firm, yellow plaques or nodules, often in a periorbital distribution. It is often accompanied by an elevated ERYTHROCYTE SEDIMENTATION RATE; LEUKOPENIA; and MONOCLONAL GAMMOPATHY (IgG-kappa type) and systemic involvement.

Also Known As:

Networked: 63 relevant articles (3 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neurologic Manifestations (Neurological Manifestations)
2.	Paraproteinemias (Monoclonal Gammopathy)
3.	Necrobiosis Lipoidica
4.	Multiple Myeloma
5.	Granuloma

Experts

1.	Ziemer, Mirjana: 2 articles (09/2010 - 09/2005)
2.	Barrett, Terry L: 2 articles (07/2004 - 05/2004)
3.	Lynch, Jane M: 2 articles (07/2004 - 05/2004)
4.	Chave, T A: 2 articles (09/2001 - 01/2001)
5.	Kindle, Scott A: 1 article (04/2015)
6.	Hart, Philip A: 1 article (04/2015)
7.	Chari, Suresh T: 1 article (04/2015)
8.	Lehman, Julia S: 1 article (04/2015)
9.	Smyrk, Thomas C: 1 article (04/2015)
10.	Pittelkow, Mark R: 1 article (04/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Necrobiotic Xanthogranuloma:

1.	Prednisolone (Predate)FDA LinkGeneric 01/01/2011 - "Letter: Necrobiotic xanthogranuloma of extremities in an elderly patient successfully treated with low-dose prednisolone." 01/01/2001 - "Recalcitrant necrobiotic xanthogranuloma responding to pulsed high-dose oral dexamethasone plus maintenance therapy with oral prednisolone." 11/01/1992 - "The patient's necrobiotic xanthogranuloma and neurologic symptoms improved after he received four 5-day courses of melphalan, 2 mg/day, and prednisolone, 20 mg/day, at 4-week intervals." 11/01/2007 - "Necrobiotic xanthogranuloma with paraproteinemia successfully treated with melphalan, prednisolone and skin graft."
2.	Melphalan (Alkeran)FDA LinkGeneric 09/01/2005 - "Necrobiotic xanthogranuloma-rapid progression under treatment with melphalan." 11/01/1992 - "The patient's necrobiotic xanthogranuloma and neurologic symptoms improved after he received four 5-day courses of melphalan, 2 mg/day, and prednisolone, 20 mg/day, at 4-week intervals." 06/01/2002 - "This case report details how melphalan and prednisone can be administered in the successful treatment of necrobiotic xanthogranuloma with lambda paraproteinemia." 06/01/2002 - "Necrobiotic xanthogranuloma with lambda paraproteinemia: case report of successful treatment with melphalan and prednisone." 11/01/2007 - "Necrobiotic xanthogranuloma with paraproteinemia successfully treated with melphalan, prednisolone and skin graft."
3.	InterferonsIBA 11/01/1999 - "Interferon alpha-2a monotherapy for necrobiotic xanthogranuloma." 05/01/2010 - "Because of the variability of presentation, there is no consensus on the best treatment for necrobiotic xanthogranuloma, which may include surgery, chemotherapy, interferon, plasmapheresis and radiation therapy."
4.	Ficusin (Psoralen)IBA 04/01/2010 - "Necrobiotic xanthogranuloma without paraproteinaemia: marked improvement with psoralen ultraviolet A treatment."
5.	Immunoglobulin G (IgG)IBA 10/01/2010 - "However, systemic disease IgG4 can be associated with necrobiotic xanthogranuloma of the orbit." 10/01/2010 - "One patient presenting with necrobiotic xanthogranuloma had signs of systemic IgG4 disease. " 10/01/2010 - "Orbital necrobiotic xanthogranuloma associated with systemic IgG4 disease." 02/01/1987 - "A case of necrobiotic xanthogranuloma is reported, occurring in a 46-year-old man and associated with an IgG kappa myeloma." 06/01/1997 - "The most recent histologic findings are those of necrobiotic xanthogranuloma, with results of laboratory studies revealing a coexistent IgG kappa paraproteinemia. "
6.	Immunoglobulins (Immunoglobulin)IBA 04/01/2015 - "It remains unclear whether the association between immunoglobulin G4-related disease and necrobiotic xanthogranuloma or other xantho(granulo)matous processes represents shared pathophysiology, a mutual underlying driver, or coincidence, though the results of this study cast doubt on the latter. " 09/01/2013 - "Successful treatment of necrobiotic xanthogranuloma with intravenous immunoglobulin." 09/01/2010 - "Successful treatment of necrobiotic xanthogranuloma with intravenous immunoglobulin." 09/01/1986 - "We speculate that increased serum immunoglobulins complexed with lipid may be deposited in the skin, leading to a foreign body giant cell reaction and the subsequent characteristic histopathologic features of necrobiotic xanthogranuloma." 09/01/2014 - "Treatment of refractory necrobiotic xanthogranulomas with extracorporeal photopheresis and intravenous immunoglobulin."
7.	Dexamethasone (Maxidex)FDA LinkGeneric 08/01/1991 - "We observed a 40-year-old woman with necrobiotic xanthogranuloma from the inception of indurated eyelid and periorbital infiltrates and concurrent stage I multiple myeloma to resolution of infiltrates in skin and bone marrow after pulsed high-dose oral dexamethasone therapy. " 06/01/2009 - "Necrobiotic xanthogranuloma successfully treated with a combination of dexamethasone and oral cyclophosphamide." 01/01/2001 - "Recalcitrant necrobiotic xanthogranuloma responding to pulsed high-dose oral dexamethasone plus maintenance therapy with oral prednisolone." 06/01/2011 - "Complete response to thalidomide and dexamethasone in a patient with necrobiotic xanthogranuloma associated with monoclonal gammopathy: a case report and review of the literature." 08/01/2005 - "Cyclophosphamide-dexamethasone pulsed therapy for treatment of recalcitrant necrobiotic xanthogranuloma with paraproteinemia and ocular involvement."
8.	Immunoglobulin A (IgA)IBA 02/01/2012 - "[Necrobiotic xanthogranuloma revealing an IgA gammapathy]." 12/01/1990 - "Necrobiotic xanthogranuloma with IgA paraproteinemia and extracutaneous involvement." 12/01/1990 - "Necrobiotic xanthogranuloma with IgA multiple myeloma: a case report and literature review." 12/01/1990 - "To our knowledge, this is the first report of an IgA type paraproteinemia and IgA type multiple myeloma associated with necrobiotic xanthogranuloma." 05/01/1995 - "Necrobiotic xanthogranuloma is associated with IgG paraproteinaemia, and occasionally with an IgA paraproteinaemia. "
9.	CholesterolIBA 02/01/1988 - "When cholesterol clefts are seen in biopsy specimens of necrobiosis, necrobiotic xanthogranuloma must be ruled out. " 02/01/1988 - "Necrobiosis lipoidica diabeticorum with cholesterol clefts in the differential diagnosis of necrobiotic xanthogranuloma." 05/01/1995 - "Necrobiotic xanthogranuloma is a rare disease marked by yellowish-brown plaques with destructive growth and a tendency to ulceration, which shows a characteristic histopathological pattern of palisading granuloma with foam cells, Touton's and foreign-body giant cells, bizarre multinuclear giant cells, cholesterol clefts and marked necrobiosis of the connective tissue. " 01/15/2001 - "A skin biopsy specimen showed a granulomatous infiltrate consisting of lymphocytes, histiocytes, plasma cells, foam cells as well as Touton and bizarre-appearing foreign-body giant cells and cholesterol clefts typical of necrobiotic xanthogranuloma besides a deposit of amyloid. " 10/01/2000 - "Histopathologic examination showed all the major features of a necrobiotic xanthogranuloma (NX) involving the entire dermis and subcutis, including areas of necrobiosis with cholesterol clefts, granulomatous infiltrate with some bizarre giant cells, numerous Touton cells, foamy cells, lymphoid follicles with germinal centers, foci of plasma cells, and "Touton cell panniculitis." A laboratory investigation revealed only slightly increased titers of cholesterol, strong positivity of anti-Borrelia antibodies, and diffuse skeletal osteoporosis with fractures of seven vertebrae. "
10.	Chlorambucil (Leukeran)FDA Link 05/01/2012 - "Characteristic clinical and histological features of necrobiotic xanthogranuloma are presented, as well as a discussion regarding management and the use of chlorambucil." 05/01/2012 - "This report of necrobiotic xanthogranuloma associated with chronic lymphocytic leukaemia describes the response of skin lesions to chlorambucil. " 05/01/2012 - "Necrobiotic xanthogranuloma: response to chlorambucil." 01/01/2006 - "We report a case of necrobiotic xanthogranuloma that responded to treatment with chlorambucil. " 01/01/2006 - "Necrobiotic xanthogranuloma treated with chlorambucil."

Therapies and Procedures

1.	Plasmapheresis 08/01/1987 - "A case of necrobiotic xanthogranuloma treated with plasmapheresis is reported. " 08/01/1987 - "Plasmapheresis, a treatment modality for necrobiotic xanthogranuloma." 08/01/1987 - "Successful healing of ulcerated lesions and lowering of the paraproteinemia was achieved by plasmapheresis in our patient with necrobiotic xanthogranuloma when cytotoxic therapy failed." 05/01/2010 - "Because of the variability of presentation, there is no consensus on the best treatment for necrobiotic xanthogranuloma, which may include surgery, chemotherapy, interferon, plasmapheresis and radiation therapy."
2.	Drug Therapy (Chemotherapy) 05/01/2010 - "Because of the variability of presentation, there is no consensus on the best treatment for necrobiotic xanthogranuloma, which may include surgery, chemotherapy, interferon, plasmapheresis and radiation therapy."
3.	Stem Cell Transplantation 04/01/2007 - "This is the first report of autologous stem cell transplantation in a patient with necrobiotic xanthogranuloma." 04/01/2007 - "Necrobiotic xanthogranuloma successfully treated with autologous stem cell transplantation."
4.	Blepharoplasty 01/01/2008 - "We present a case report of necrobiotic xanthogranuloma (NXG) in a 76-year-old Caucasian lady occurring as a nodule in a blepharoplasty scar. "
5.	Transplants (Transplant) 11/01/2007 - "Necrobiotic xanthogranuloma with paraproteinemia successfully treated with melphalan, prednisolone and skin graft."