Symptoma

Necrobiotic Xanthogranuloma

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]

  • Recurrent Respiratory Infections

    Case Report A 17-year-old girl, with history of recurrent respiratory infections and obstructive pulmonary disease, was admitted to our institution for the management of cough and respiratory failure. [avensonline.org]

  • Renal Artery Stenosis

    The patient's renal impairment was unrelated to NXG; it was due to bilateral renal artery stenosis, a rare complication of renal denervation. She underwent bilateral renal artery stenting and her renal function improved. [mja.com.au]

  • 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]

  • Blurred Vision

    He denied pain, diplopia, tearing, and blurred vision. His medical history included asymptomatic annular plaques on the trunk and extremities for at least a decade. Ophthalmological examination revealed a necrotizing scleritis of the right eye. [ncbi.nlm.nih.gov]

    vision, double vision, bulging of the eyeball, drooping of the eyelid, and restricted eye movement. [2] In most cases, other parts of the body, such as the trunk, legs, face, and arms, are also involved. [rarediseases.info.nih.gov]

    Signs and symptoms: Approximately 50% of people with necrobiotic xanthogranuloma have eyes problems, such as burning or itching of the eye, blurred vision, double vision, reduced vision, drooping of the eyelid, bulging of the eyeball, and restricted eye [xpertdox.com]

  • Periorbital Edema

    Periorbital edema and plaques improved during a 6-month regimen of acitretin but returned to baseline just months after discontinuation. [ncbi.nlm.nih.gov]

    Dermatologic examination revealed substantial periorbital edema with multiple yellow indurated plaques (Figure). There were no additional findings on physical examination. Bilateral periorbital edema with yellow indurated plaques (A). [mdedge.com]

    Examination revealed yellow, firm papules coalescing into plaques causing cicatricial lagophthalmos, with surrounding erythema, and bilateral periorbital edema (Fig. 1 ). [karger.com]

  • Myelopathy

    He was a human T-lymphotropic virus type 1 (HTLV-1) carrier who had a high titer of circulating anti-HTLV-1 antibodies and neurologic abnormalities that suggested HTLV-1-associated myelopathy. [ncbi.nlm.nih.gov]

    […] disorder *angiopathy 【血管神経膠腫】*angioglioma 【血管神経膠腫症】*angiogliomatosis 【血管神経症】*vasoneurosis *angioneuropathy 【血管心臓炎】*angiocarditis 【血管性耳炎】*angiotitis ( 耳血管炎) 【血管性水腫】*vascular edema 【血管性痴呆】*vascular dementia 【血管性母斑】*vascular nevus 【血管性ミエロパチー】*vascular myelopathy [medo.jp]

  • Confusion

    Abstract Necrobiotic xanthogranuloma with paraproteinemia is a progressive and destructive process that is often confused both clinically and histologically with other granulomatous and xanthomatous entities. [ncbi.nlm.nih.gov]

  • 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]

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]

  • Lymphopenia

    During 6 years of follow-up, anemia, neutropenia with marked lymphopenia, and increased ESR were found, while serum cholesterol and triglyceride levels decreased from hyper to hypo values. [ncbi.nlm.nih.gov]

    She also had raised ESR, IgG- κ paraproteinaemia, neutropenia, lymphopenia, and low complement C4. Figure 1 Necrobiotic xanthogranuloma on the skin of the right thigh. Visual acuities were 6/18 right eye and 6/5 left eye. [nature.com]

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 Associations [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]

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]

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]

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]