Nekrobiotisches Xanthogranulom mit Haut- und Leberamyloidose

Westermann, G.; August, Christian; Bonsmann, Gisela; Rahn, K. H.; Kisters, Klaus

doi:10.1007/pl00002153

Cited by 8 publications

(3 citation statements)

References 22 publications

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“…Histological changes of NXG are prominent in the skin, but some features have been observed in visceral organs. Although associated hepatomegaly has been observed, 2 histological examination of the liver has only been reported in a few cases — two patients showed reactive changes including nodular transformation, 4 , 6 and one showed amyloidosis 7 . Syncytial giant cell hepatitis was reported in a patient with NXG, but there was no more than a tenuous association 8 .…”

Section: Discussionmentioning

confidence: 99%

A multisystem granulomatous disease: necrobiotic xanthogranuloma with hepatic involvement

Santosaputri

Ellis

Nagiah

et al. 2014

Medical Journal of Australia

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Necrobiotic xanthogranuloma is a rare, multisystem granulomatous disease that can have skin and visceral organ involvement. It is strongly associated with paraproteinaemia, and has rare potential for transformation to myeloma or lymphoma, warranting ongoing surveillance. We report a challenging case of necrobiotic xanthogranuloma, believed to be the fi rst reported case of hepatic involvement. Clinical recordA 51-year-old woman was referred to hospital after an incidental fi nding of abnormal liver function test results . She was otherwise well and asymptomatic. Her past medical history included resistant hypertension with renal denervation 8 months earlier, and chronic macrocytic anaemia secondary to alcohol. Physical examination was largely unremarkable apart from several skin nodules and hepatomegaly (4 cm below the costal margin). She underwent an abdominal ultrasound, which showed multiple hypoechoic liver lesions suspicious for metastasis. An abdominal computed tomography (CT) scan (Box 1) confi rmed the presence of hypovascular liver lesions suggestive of metastasis. Malignancy screening, including mammography, colonoscopy and CT of the chest, abdomen and pelvis, was negative. A subsequent liver biopsy specimen showed necrotising granulomas with no evidence of malignancy. The initial histopathological impression was of mycobacterial infection, despite negative Ziehl-Neelsen and Gram stains and the absence of infective organisms.Further assessment found no risk factors for tuberculosis exposure and no symptoms of underlying infection. Dermatology consultation was requested and the skin nodules were closely examined. These included a 1 2 cm violaceous nodule on the left side of the woman's neck, a 2 5 cm yellowish plaque on her left fl ank, and a large violaceous-yellow plaque exhibiting Koebner phenomenon along the scar from her left total knee replacement surgery (Box 2). The patient reported that they had been present for 8-12 months and were asymptomatic. Biopsy specimens from the lesions on her neck and fl ank showed identical histological features to the liver specimen, again negative for Mycobacterium tuberculosis on Ziehl-Neelsen stain, culture and polymerase chain reaction (PCR) testing. A previous biopsy sample of the plaque overlying her kneereplacement scar had been taken by a dermatologist and showed non-discrete granulomatous infl ammation and necrobiosis (Box 3, A). In retrospect, this was consistent with her current skin sample.Thorough investigations were performed for causes of systemic granulomatous diseases. Infective causes that were investigated included histoplasmosis, brucellosis and mycobacterial infections. However, results from extensive serological testing, cultures, tuberculosis PCR testing of the skin and liver biopsy samples, and an interferon-γ release assay were negative. Non-infective differential diagnoses included sarcoidosis or vasculitis. However, angiotensin-converting enzyme concentration was low and no hilar lymphadenopathy was found on CT of the chest. Results of au...

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Section: Discussionmentioning

confidence: 99%

A multisystem granulomatous disease: necrobiotic xanthogranuloma with hepatic involvement

Santosaputri

Ellis

Nagiah

et al. 2014

Medical Journal of Australia

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“…Diversos órgãos internos podem ser acometidos, nomeadamente o pulmão, coração, fígado, laringe, faringe, músculo esquelético, rim, baço, ovário e intestino 1,2,5 . Do nosso conhecimento, o envolvimento hepático no contexto de XN foi reportado na literatura em quatro casos, um sob a forma de amiloidose hepática, dois como nódulos hepáticos e um outro como hepatite de células gigantes 3,[7][8][9] . Na nossa doente observamos esta última associação e, tal como proposto por Amer et al, as duas condições poderão ter uma etiologia comum 3 , dada a similitude patogénica.…”

Section: Discussionunclassified

Xantogranuloma Necrobiótico Associado a Hepatite De Células Gigantes

Ferreira

Mota

Baudrier

et al. 2013

SPDV

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Descrevemos uma doente de 44 anos com episódios recorrentes de olho vermelho, mialgias, hipercoles- terolemia, hipocomplementemia, gamapatia monoclonal e hepatite de células gigantes que foi referenciada à nossa consulta por placa eritemato-violácea do braço direito. A biópsia cutânea permitiu o diagnóstico de xantogranuloma necrobiótico. Este caso ilustra a importância da correcta caracterização das lesões cutâneas num contexto de uma doença sistémica de difícil abordagem e evidencia o segundo caso relatado de associação do xantogranuloma ne- crobiótico com hepatite de células gigantes.PALAVRAS-CHAVE – Xantogranuloma necrobiótico; Hepatite células gigantes; Gamapatia monoclonal.

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“…There is no gender preference 14,19 . NXG lesions have been described in patients with scleroderma, 20 asthma, and systemic amyloidosis 21,22 . Although NXG lesions are typically periorbital, cases lacking periorbital lesions have been reported 19,23,24 …”

Section: Necrobiotic Xanthogranulomamentioning

confidence: 99%

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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Nekrobiotisches Xanthogranulom mit Haut- und Leberamyloidose

Abstract: Apart from treatment of skin lesions and ophthalmic manifestations further investigations are necessary because necrobiotic xanthogranuloma can be associated with malignancy and can involve internal viscera like lungs, heart and liver.

Cited by 8 publications

References 22 publications

A multisystem granulomatous disease: necrobiotic xanthogranuloma with hepatic involvement

A multisystem granulomatous disease: necrobiotic xanthogranuloma with hepatic involvement

Xantogranuloma Necrobiótico Associado a Hepatite De Células Gigantes

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

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