Nekrobiotisches Xanthogranulom bei IgG-kappa-Plasmozytom und Quincke-�dem

Hafner, Oliver; Witte, Torsten; Schmidt, Reinhold; Vakilzadeh, F

doi:10.1007/s001050050080

Cited by 11 publications

(5 citation statements)

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“…Decreased levels of C1 inhibitor have also been reported in NXG patients and are thought to be due to autoantibodies against the circulating paraprotein associated with NXG, which becomes deposited in the skin. The deposited paraprotein triggers immune complex formation, causing C1 inactivator to be consumed 31 . The deposited paraprotein may also have functional features of a lipoprotein and therefore may bind to lipoprotein receptors of the histiocytes, inducing xanthoma formation 32…”

Section: Necrobiotic Xanthogranulomamentioning

confidence: 99%

“…The deposited paraprotein triggers immune complex formation, causing C1 inactivator to be consumed. 31 The deposited paraprotein may also have functional features of a lipoprotein and therefore may bind to lipoprotein receptors of the histiocytes, inducing xanthoma formation. 32 Another theory of NXG pathogenesis is that the paraproteinemia of the disease is the primary phenomenon, with a secondary proliferation of macrophages with receptors for the Fc portion of IgG 29 .…”

Section: Nxg -Clinicalmentioning

confidence: 99%

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Collagenolytic (necrobiotic) granulomas: part II – the ‘red’ granulomas

Lynch

Barrett

2004

J Cutan Pathol

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A collagenolytic or necrobiotic non-infectious granuloma is one in which a granulomatous infiltrate develops around a central area of altered collagen and elastic fibers. The altered fibers lose their distinct boundaries and exhibit new staining patterns, becoming either more basophilic or eosinophilic. Within the area of altered collagen, there may be deposition of acellular substances such as mucin (blue) or fibrin (red), or there may be neutrophils with nuclear dust (blue), eosinophils (red), or flame figures (red). These color distinctions can be used as a simple algorithm for the diagnosis of collagenolytic granulomas, i.e. 'blue' granulomas vs. 'red' granulomas. Eight diagnoses are included within these two groupings, which are discussed in this two-part article. In the previously published first part, the clinical presentation, pathogenesis and histologic features of the 'blue' collagenolytic granulomas were discussed. These are the lesions of granuloma annulare, Wegener's granulomatosis, and rheumatoid vasculitis. In this second half of the series, the 'red' collagenolytic granulomas are discussed; these are the lesions of necrobiosis lipoidica, necrobiotic xanthogranuloma, rheumatoid nodules, Churg-Strauss syndrome, and eosinophilic cellulitis (Well's Syndrome).Lynch JM, Barrett TL. Collagenolytic (necrobiotic) granulomas: part II -the 'red' granulomas.

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Section: Necrobiotic Xanthogranulomamentioning

confidence: 99%

Section: Nxg -Clinicalmentioning

confidence: 99%

Collagenolytic (necrobiotic) granulomas: part II – the ‘red’ granulomas

Lynch

Barrett

2004

J Cutan Pathol

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“…Iritis and uveitis have also been reported. Moreover, NXG is associated with monoclonal gammopathy and, often, plasma proliferative disorders including multiple myeloma [1,5,6,8]. A case of a woman with periorbital lesions is reported.…”

Section: Introductionmentioning

confidence: 96%

Necrobiotic xanthogranuloma of the eyelids with paraproteinemia

Bortolani

Barisoni

Bontempini³

1999

European Journal of Plastic Surgery

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Necrobiotic xantogranuloma (NXG) with paraproteinemia is characterized by multiple nodules or plaques that involve the periorbital area. The lesions frequently become inflamed leading to superficial ulceration; multiple myeloma is present in some patients. A case of NXG with IgG monoclonal gammopathy is reported; the ocular symptoms were clinically pre-eminent. Histologically the granulomas are characterized by collagen necrobiosis, many foamy histiocytes, Touton giant cells, bizarre foreign body cells, and were positive for light chain kappa. In this patient, chemotherapy did not have a favorable response, therefore the lesions were excised and the resulting defects were covered with split skin grafts. The patient died seven months after surgical treatment, this was the result of complications of myeloma.

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“…Die assoziierte Paraproteinämie kann persistieren, oder es kann sich ein manifestes Plasmozytom entwickeln. In einem Fall wurde auch ein im Verlauf entstandenes MDS mitgeteilt [16] gen, chronisch lymphatischer Leukämie, Diabetes mellitus, nephrotischem Syndrom bei Amyloidose der Niere, COPD, Lungenfibrose, Kardiomyopathie, arterieller Hypertonie, QuinckeÖdem (durch Verminderung des C1-Inhibitors, siehe oben), Polyneuropathie, Morbus Basedow, Prolactin sezernierendem Hypophysenadenom, juveniler Arthritis, Magen-, Lungen-, Rektum-und Zervixkarzinom berichtet [3,4,10,16,17,20,26]. EKG-Verände-rungen wie AV-Block, linksanteriorer Hemiblock, abnorme T-Wellen und echokardiographische Veränderungen wie linksventrikuläre Hypertrophie und ventrikuläre Funktionsstörungen wurden nachgewiesen [26].…”

Section: Introductionunclassified

“…mit Lipiden verbinden und sich diese Komplexe in der Haut ablagern und so zu einer Nekrose und Fremdkörperreaktion führen [1,10], wobei in unserem Fall Ablagerungen von Leichtkettenamyloid als Auslöser angenommen werden könnten. Eine aktuelle japanische Untersuchung fand heraus, dass eine beschleunigte Komplementreaktion und erhöhte M-CSF-(Macrophage Colony Stimulating Factor-)Spiegel zu einer übermäßigen Cholesterylester-und LDL-(Low Density Lipoprotein-) Aufnahme von Monozyten führen, wodurch es dann zu einer Xanthomatose des Gewebes und außerdem zu einer Hypocholesterinämie kommt [15].…”

Section: Introductionunclassified