Necrobiotic xanthogranuloma with paraproteinaemia

Flann, S.; Wain, E. Mary; Halpern, S; Andrews, V.; Whittaker, Sean

doi:10.1111/j.1365-2230.2005.02042.x

Cited by 40 publications

(37 citation statements)

References 11 publications

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“…In our patient, bortezomib combined with low-dose systemic corticosteroids normalized the paraproteinemia but had no effect on the skin lesions. The latter improved after high-dose dexamethasone therapy, as previously reported in the literature [10, 11, 12]. This may underline that NXG is a reactive rather than a neoplastic skin disease.…”

Section: Discussionsupporting

confidence: 83%

Necrobiotic Xanthogranuloma in a Patient with Multiple Myeloma

et al. 2016

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An 82-year-old woman presented with a 9-month history of multiple, well-defined skin lesions on her neck and upper chest, progressively increasing in size. Histological examination of a skin biopsy showed a regular epidermis. In the dermis, granulomatous changes with central necrobiosis were found which extended focally into the subcutaneous fat. The necrobiotic areas were surrounded by Touton cells and foreign-body giant cells. Laboratory analysis revealed leucopenia. Serum electrophoresis and immunofixation disclosed the presence of an IgG-λ paraprotein. Bone marrow aspiration cytology showed 20% plasmatic cells. The skeletal X-ray revealed frontal and occipital osteolytic skull lesions as well as pubic osteolysis. Urinalysis was unremarkable without proteinuria. Based on the clinical, laboratory, and histological findings, necrobiotic xanthogranuloma in association with multiple myeloma was diagnosed. The patient was treated with bortezomib combined with oral dexamethasone. Follow-up at week 13 revealed the complete disappearance of the IgG paraproteinemia. However, the skin lesions remained unchanged. Therefore, bortezomib treatment was discontinued, and systemic corticosteroids were continued at a higher dosage. After the steroid treatment, the lesions markedly flattened.

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Section: Discussionsupporting

confidence: 83%

Necrobiotic Xanthogranuloma in a Patient with Multiple Myeloma

et al. 2016

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“…Necrobiotic xanthogranuloma, a rare chronic disorder prevailing in the sixth decade, occurs in association with IgG monoclonal gammopathy (usually k subtype) in about 80% of patients. Although bone marrow examination may show plasmocytosis, true multiple myeloma is not frequent (10%) 44–46 . Cryoglobulinemia occurs in 8% of case.…”

Section: Associated Disordersmentioning

confidence: 99%

The histological and pathogenetic spectrum of cutaneous disease in monoclonal gammopathies

2008

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The dermatological disorders associated with monoclonal gammopathies are clinically heterogeneous and may be divided into four groups with distinctive pathogenetic mechanisms (a) specific (infiltrative) disorders including primary and secondary cutaneous plasmacytoma and cutaneous lymphoplasmacytic infiltration of Waldenström's disease (b) skin disorders because of the deposition of monoclonal immunoglobulin (M protein), including amyloidoisis macroglobulinemia cutis, light chain deposition of Randall's disease, follicular spicules of the nose, and cryoglobulinemia (c) skin disorders associated with monoclonal gammopathies, including highly associated (>50%), weakly associated (<50%) or anecdotal and (d) miscellaneous (non specific). In most cases, histopathology is crucial to confirm or to diagnose those skin conditions and is also very useful to understand their pathogenetic mechanisms.

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“…NXG represents a rare disease of unknown origin characterized by indurated, erythematous to yellowish papules and plaques with preferential involvement of the periorbital area , but affecting also the trunk and extremities . It is frequently associated with paraproteinemia , especially IgG kappa light‐chain monoclonal gammopathy , which, in some cases, progresses to multiple myeloma .…”

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confidence: 99%