Clinically, the patient presented with dry eyes and substantial periorbital edema with multiple yellow indurated plaques. He developed the condition 30 years prior to presentation at which time it was initially diagnosed as xanthelasma. [unboundmedicine.com]

• Lymphedema

  If it's not working properly, fluid builds in your tissues and causes swelling, called lymphedema. Other lymphatic system problems can include infections, blockage, and cancer. [icdlist.com]

  […] lymph varix 【リンパ球形質細胞性リンパ腫】*lymphoplasmacytic lymphoma 【リンパ球減少症】*lymphocytopenia 【リンパ球性白血病】*lymphocytic leukemia 【リンパ組織炎】*lymphatitis 【リンパ芽球性リンパ腫】*lymphoblastic lymphoma 【リンパ腫】*lymphoma 【リンパ上皮腫】*lymphoepithelioma 【リンパ上皮性嚢胞】*lymphoepithelial cyst 【リンパ水腫】*lymphedema [medo.jp]

• Ulcer

  → proceed | ↑ close CASE REPORT A 64-year-old gentleman presented with a six-year history of non-healing cutaneous ulcers. [jddonline.com]

  Abstract Necrobiotic xanthogranuloma (NXG) is an indolent non-Langerhans cell histiocytosis characterized by yellow xanthomatous plaques that tend to ulcerate. [unboundmedicine.com]

  We have documented an unusual case of necrobiotic xanthogranuloma in a 58-year-old man with a history of recalcitrant and chronic arm and leg ulcerations in the absence of characteristic periorbital involvement. [ncbi.nlm.nih.gov]

  […] xanthogranuloma Disease definition Necrobiotic xanthogranuloma is a rare, chronic and progressive, non-Langerhans cell histiocytosis disease typically characterized by multiple, indurated, asymptomatic to pruritic, yellow-orange plaques or nodules that tend to ulcerate [orpha.net]

• Skin Lesion

  Time to development of associated malignancy ranged from 8 years before the skin lesions to 11 years after the skin lesions. Overall survival was 100% at 10 years and 90% at 15 years (95% confidence limit, 0.73 to 1.00). [ncbi.nlm.nih.gov]

Systemic workup demonstrated paraproteinemia consistent with necrobiotic xanthogranuloma and a complete blood count consistent with leukemia. [uthealth.influuent.utsystem.edu]

Progress logically from the histologic pattern, through the appropriate workup, around the pitfalls, to the best diagnosis. [books.google.com]

After the hematologic and oncologic workup was completed, intravenous immunoglobulin and acitretin were recommended to the patient as viable treatment options to reduce the cutaneous sequelae of NXG. [mdedge.com]

NXG Treatment Patients diagnosed with NXG should be referred to a hematologist-oncologist for a workup (potentially including serum protein immunoelectrophoresis, flow cytometry, and/or bone marrow biopsy). [aao.org]

• Leukopenia

  Laboratory studies showed mild leukopenia and a monoclonal gammopathy of the IgG lambda type on serum protein immunoelectrophoresis. [ncbi.nlm.nih.gov]

  It is often accompanied by an elevated ERYTHROCYTE SEDIMENTATION RATE; LEUKOPENIA; and MONOCLONAL GAMMOPATHY (IgG-kappa type) and systemic involvement. [rgd.mcw.edu]

  Low complement levels, anemia, and leukopenia are frequently present. Bone marrow examination shows plasma cell proliferation and, rarely, true myeloma. [jamanetwork.com]

  […] adenopathy(2), uveitis(2), sinus NXG(1 ), pelvic wall NXG( 1 ), cardiac NXG( 1); Laboratory findings: elevated ESR(8/10), +ANA(7/13), +RF(4/13), cryoglobulins(2/J3), low CH50(5/12), low C4(6/l1), elevated cholesterol(7/12), elevated triglyceride(3/12), leukopenia [mayoclinic.pure.elsevier.com]

• Foam Cell

  Not only subcutaneous involvement, but also, evident zones of degenerated collagen, foam cells, and cholesterol clefts were missing. Moreover, the case shows overlaps with recently published granulomatous scleromyxedema. [ncbi.nlm.nih.gov]

  Foam cells, large numbers of necrotic inflammatory cells and cholesterol clefts are absent. Atypical giant cells are not prominent. Panniculitis is localized to the septa. In necrobiotic xanthogranuloma the lobules are extensively involved. [histopathology-india.net]

  The histologic features of well-developed lesions are suggestive of the disorder and consist of diffuse dermal aggregates of foam cells with interspersed Touton-type wreath giant cells (8). [mussenhealth.us]

  Histologically, the lesions are characterized by an inflammatory infiltrate of foam cells and Touton-type multinucleated giant cells. The xanthomatized histiocytes are CD68 +, S100 – and CD1a –. [actasdermo.org]

  The distinctive histopathologic features of NXG consist of large bands of necrobiosis and a pattern of palisading histiocytic granulomas comprised of Touton giant cells, bizarre foreign body giant cells, foam cells, and cholesterol clefts. 8 These histopathologic [mdedge.com]

• Hepatocellular Carcinoma

  ◎結節性再生性過形成：nodular regenerative hyperplasia 部分的結節化：partial nodular transformation 大再生結節：large regenerative nodule 腺腫様過形成：adenomatous hyperplasia 異型腺腫様過形成：atypical adenomatous hyperplasia 初期の高分化型肝細胞癌：early hepatocellular carcinoma of well differentiated [medical-e.net]

  【肝外胆管閉塞】*extrahepatic biliary obstruction 【肝芽細胞腫】【肝芽腫】*hepatoblastoma 【肝癌】*hepatocarcinoma (＝肝細胞癌) 【肝頚静脈逆流】*hepatojugular reflux 【肝硬変症】*liver cirrhosis/*hepatic cirrhosis/*cirrhosis *hepatocirrhosis 【肝細胞癌】*hepatocellular carcinoma (＝肝癌) 【肝細胞性黄疸】*hepatocellular [medo.jp]

treatment', both in English and German, about 'human' subjects and published between 1980 and 2014, documenting adequate treatment for NXG. [ncbi.nlm.nih.gov]

NXG prognosis is poor with several treatments showing variable results. Treatment of monoclonal gammopathy with alkylating agents does not necessarily influence the activity of the skin disease and vice versa. [ncbi.nlm.nih.gov]

The prognosis for NXG is good, but no curative treatment is available. [scmsjournal.com]

The overall prognosis is favorable with the exception of those patients who develop hematopoetic malignancy. The dermatologie manifestations may pursue three clinical courses: spontaneous resolution; remain persistent; or rarely, progress. [mussenhealth.us]

Necrobiotic Xanthogranuloma Lethal Non-Langerhans Cell Histiocytoses: Necrobiotic Xanthogranuloma and Xanthoma Disseminatum ■ Synonyms: NXG—Necrobiotic xanthogranuloma with paraproteinemia XD—Montgomery's syndrome ■ Etiology: NXG—Unknown XD—Unknown ■ [mussenhealth.us]

McKee's Pathology of the Skin is the most complete, in-depth resource on dermatopathology, covering etiology, pathogenesis, disease mechanisms, and recent genetic, molecular, and basic science data. Drs. J. [books.google.es]

Due to the rarity and uncertain etiology, there are no definitive first-line therapies. Necrobiotic xanthogranuloma (NXG) was first described in 1980 by Kossard and Winkelmann 1 as a xanthomatosis associated with paraproteinemia. [mdedge.com]

Etiology Pathophysiology The pathogenesis of NXG is poorly understood. [dermatologyadvisor.com]

ABSTRACT:Cortexolone 17α-propionate (clascoterone) is a novel topical androgen antag... more Skin Cancer Epidemiology and Sun Protection Behaviors Among Native American.... [jddonline.com]

Discussion Epidemiology Limbal AOX is extremely rare [23], and like JXG in children, it occurs predominantly in males [25]. Eight of 11 cases in this review affected male patients. [slideheaven.com]

This review focuses on noninfectious granulomatous conditions, with particular emphasis on age-related differences in the onset, epidemiology, clinical manifestations, prognosis, and age-specific management of specific granulomatous disorders. [pubfacts.com]

The pathophysiology underlying NXG remains unknown although the involvement of MIg is suspected. [ncbi.nlm.nih.gov]

As more cases are reported, we hope to better understand the pathophysiology and treatment of this disorder. References 1. hen KY, Leslie W, Mahon B, Venugopal P. [hematologyandoncology.net]

Etiology Pathophysiology The pathogenesis of NXG is poorly understood. [dermatologyadvisor.com]

Science ‎ Pagina 305 - Silva P (1994) Effects of saline, mannitol, and furosemide to prevent acute decreases in renal function induced by ‎ Pagina 465 - Bennett NT, Schultz GS (1993) Growth factors and wound healing: part II. [books.google.it]

Funding Sources This study was supported by an unrestricted grant from the Research to Prevent Blindness (New York, NY, USA), and the Lions and Lionesses of Minnesota. [karger.com]

Spontaneous regression is unlikely, and thus prompt surgical intervention should be considered to prevent sight-threatening complications. [slideheaven.com]

Am J Dermatopathol 2016 Jun;38(6):468-70 *Department of Biomedicine and Prevention, Institute of Anatomic Pathology, University of Rome Tor Vergata, Rome, Italy †Unit of Dermatopathology, San Gallicano Dermatological Institute, IRCCS Rome, Rome, Italy [pubfacts.com]