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Necrobiotic Xanthogranuloma

Presentation

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. [ncbi.nlm.nih.gov]

Approximately 30 years prior to the current presentation, the patient presented to a dermatologist with dry eyes and periorbital cutaneous lesions that were originally diagnosed as xanthelasma. [mdedge.com]

Respiratoric

  • Sputum

    *hemosputum *sputum cruentum[L] 【血鉄症】*hemosiderosis 【血糖症】*glycemia 【血尿】*hematuria *erythruria [赤血球尿症] *urina cruenta[L] 【血友病】*hemophilia 【血瘤】*hematocele 【欠指症】【欠趾症】*ectrodactyly/*ectrodactylia/*ectrodactylism 【欠肢症】*ectromelia 【欠肢体】*ectromelus 【欠乏症】*deficiency [medo.jp]

Jaw & Teeth

  • Parotid Mass

    RESULTS: A 48-year-old man presented with a right parotid mass. Fine needle aspiration cytology was suggestive of Warthin's tumour, for which the patient underwent a subtotal parotidectomy. [ncbi.nlm.nih.gov]

Neurologic

  • Generalized Tonic-Clonic Seizure

    This 52-year-old man, in whom NXG with all its cutaneous manifestations had been diagnosed, presented with three episodes of generalized tonic-clonic seizures. [ncbi.nlm.nih.gov]

Workup

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]

Treatment

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

Prognosis

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]

Etiology

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]

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]

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]

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

Epidemiology

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

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]

Pathophysiology

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]

Prevention

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]

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]

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]

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

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