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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]
  • Abstract Necrobiotic xanthogranuloma (NXG) usually shows a stereotypical histopathologic presentation. However, few unusual cases have been published. We present a patient with NXG showing exceptional histopathologic features.[ncbi.nlm.nih.gov]
  • We also review the clinical presentations and treatments for this rare condition. METHOD: Case report and review of necrobiotic xanthogranuloma. RESULTS: A 48-year-old man presented with a right parotid mass.[ncbi.nlm.nih.gov]
  • Dense sheets of foamy histiocytes were present in the dermis and contained foci of necrobiosis. Numerous Touton giant cells were present and cholesterol clefts were prominent. Special stains for organisms were negative.[ncbi.nlm.nih.gov]
  • Abstract A uniquely indolent case of necrobiotic xanthogranuloma with bilateral, periorbital involvement was presented. This patient presented with cutaneous eyelid lesions of 20 years' duration.[ncbi.nlm.nih.gov]
Asymptomatic
  • The lesions were asymptomatic. These were found to be consistent with the diagnosis of Necrobiotic Xanthogranuloma (NXG) on histology.[ncbi.nlm.nih.gov]
  • Homepage Rare diseases Search Search for a rare disease Necrobiotic xanthogranuloma Disease definition Necrobiotic xanthogranuloma is a rare, chronic and progressive, non-Langerhans cell histiocytosis disease typically characterized by multiple, indurated, asymptomatic[orpha.net]
  • Abstract We report on a 60-year-old overweight white woman who presented with an asymptomatic flat, hard, yellow-brown subcutaneous plaque on her right hip. A total excision was performed.[ncbi.nlm.nih.gov]
  • Most cases are asymptomatic, and oral mucosal erosions are sometimes present. Ophthalmologic complications are observed in many patients, and NXG may involve other extracutaneous sites.[ncbi.nlm.nih.gov]
  • 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]
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]
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]
Diplopia
  • Case 3 had a more typical presentation of diplopia and blepharoptosis caused by orbital and periorbital infiltrative masses. Case 4 had nondeforming periocular skin lesions over a 6-year period.[ncbi.nlm.nih.gov]
  • 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]
  • They include burning, itching, blurred vision, diplopia and acute transient loss, periocular lesions, lesions in the anterior orbit, proptosis, blepheroptosis and restricted ocular motility.[ 1 – 3 ] Our patient had ocular findings in the form of lid[ncbi.nlm.nih.gov]
  • If there is deeper extension of the lesions into the orbit, patients may report blurred vision, diplopia, or transient vision loss and may develop proptosis, strabismus, scleritis, keratitis, iritis, and conjunctivitis. 1,3,4 Since the first description[aao.org]
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]
  • 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[xpertdox.com]
  • They include burning, itching, blurred vision, diplopia and acute transient loss, periocular lesions, lesions in the anterior orbit, proptosis, blepheroptosis and restricted ocular motility.[ 1 – 3 ] Our patient had ocular findings in the form of lid[ncbi.nlm.nih.gov]
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]
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]
Mediastinal Mass
  • He will continue to be followed for progression of disease or symptoms caused by the anterior mediastinal mass.[hematologyandoncology.net]
Lymphocytopenia
  • […] pneumonia 【リノスポリジウム症】*rhinosporidiosis 【リポイド類壊死症】*necrobiosis lipoidica[L] ( 類脂質性類壊死症) 【リンパ陰嚢】*lymph scrotum *elephantiasis scroti[L] 【リンパ管炎】*lymphangitis 【リンパ管腫】*lymphangioma 【リンパ管怒張】【リンパ管瘤】*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[medo.jp]

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]
  • This combined treatment resulted in flattening of the NXG lesions and a reduction of symptomatic pruritus within the treatment zone.[ncbi.nlm.nih.gov]
  • The rationale for this treatment was the successful use of PUVA treatment in other necrobiotic conditions such as granuloma annulare and necrobiosis lipoidica.[ncbi.nlm.nih.gov]
  • The treatment of these lesions represents a true challenge for the clinician and palliative treatment of cutaneous lesions of NXG with CO(2) laser may constitute an alternative treatment in selected cases.[ncbi.nlm.nih.gov]
  • Additional treatment options include corticosteroids, intravenous immunoglobulin, lenalidomide, interferon, radiation therapy, and surgery. In some cases, the symptoms of NXG may return after treatment.[rarediseases.info.nih.gov]

Prognosis

  • This disease is a chronic process, and a patient's prognosis depends on the degree of extracutaneous involvement and the presence of visceral malignancies.[ncbi.nlm.nih.gov]
  • The prognosis for NXG is good, but no curative treatment is available.[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 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 Associations[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]

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]
  • Allergo Journal 4:58 (1995) Abeck D., Ring J.: Epidemiology and prevalance of atopic dermatitis.[haut-allergie-nymphenburg.de]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology underlying NXG remains unknown although the involvement of MIg is suspected.[ncbi.nlm.nih.gov]
  • Abstract Necrobiotic xanthogranuloma (NXG) is a rare dermatosis with a poorly understood pathophysiology. Studies comparing treatments for such lesions are limited.[ncbi.nlm.nih.gov]
  • Based on this pathophysiology, we elected to start systemic glucocorticoids, titrated to clinical and metabolic response.[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]
  • .: Systemic vitamin C and vitamin E do not prevent photoprovocation test reactions in polymorphous light eruption.[haut-allergie-nymphenburg.de]

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