Necrobiotic xanthogranuloma with multiple myeloma

Bhari, Neetu; Chiramel, Minu Jose; Vedi, Kamal Kumar; Nath, Devajit; Sandip, Sonia; Kumar, Rajive; Kumar, Lalit; Sharma, Vinod; Sethuraman, Gomathy

doi:10.1111/ced.12620

Cited by 8 publications

(5 citation statements)

References 4 publications

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“…In the systematic review, the initial search yielded 1199 records, 151 of which were included (Figure). These reports described 201 patients with NXG, with demographics, comorbidities, and clinical features (detailed in the eTable in the Supplement).…”

Section: Resultsmentioning

confidence: 89%

A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria

et al. 2020

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IMPORTANCE Necrobiotic xanthogranuloma (NXG) is a non-Langerhans cell histiocytosis classically associated with paraproteinemia attributable to plasma-cell dyscrasias or lymphoproliferative disorders. Despite the morbidity of NXG, the literature is limited to case reports and small studies, and diagnostic criteria are lacking.OBJECTIVE To evaluate the characteristics of NXG and propose diagnostic criteria. DESIGN, SETTING, AND PARTICIPANTSThis multicenter cross-sectional study was conducted at tertiary academic referral centers and followed by a systematic review and a consensus exercise. The multicenter cohort included patients with NXG diagnosed at the Brigham and Women's and Massachusetts General Hospitals (2000-2018), the University of Iowa Hospitals and Clinics (2000-2018), and the University of Pennsylvania Health System (2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016)(2017)(2018). The systematic review was conducted in 2018 and included patients with NXG identified in the Cochrane, Ovid EMBASE, PubMed, and Web of Science databases. The consensus exercise was conducted by 8 board-certified dermatologists to identify diagnostic criteria.MAIN OUTCOMES AND MEASURES Demographic factors, comorbidities, clinical features, and treatment response. RESULTSOf 235 included patients with NXG (34 from the multicenter cohort and 201 from the systematic review results), the mean (SD) age at presentation was 61.6 (14.2) years; 147 (62.6%) were female. Paraproteinemia was detected in 193 patients (82.1%), most often IgG-κ (117 patients [50.0%]). A malignant condition was detected in 59 patients (25.1%), most often multiple myeloma (33 patients [14.0%]). The overall rate of paraproteinemia and/or a malignant condition was 83.8% (197 patients). In the multicenter cohort, evolution of paraproteinemia into multiple myeloma was observed up to 5.7 years (median [range], 2.4 [0.1-5.7] years) after NXG presentation. Cutaneous lesions consisted of papules, plaques, and/or nodules, typically yellow or orange in color (113 of 187 [60.4%]) with a periorbital distribution (130 of 219 [59.3%]). The eye was the leading site of extracutaneous involvement (34 of 235 [14.5%]). In the multicenter cohort, intravenous immunoglobulin had the best treatment response rate (9 of 9 patients [100%]), followed by antimalarial drugs (4 of 5 patients [80%]), intralesional triamcinolone (6 of 8 patients [75%]), surgery (3 of 4 patients [75%]), chemotherapy (8 of 12 patients [67%]), and lenalidomide or thalidomide (5 of 8 patients [63%]). The consensus exercise yielded 2 major criteria, which were (1) clinical and (2) histopathological features consistent with NXG, and 2 minor criteria, consisting of (1) paraproteinemia, plasma-cell dyscrasia, and/or other associated lymphoproliferative disorder and (2) periorbital distribution of cutaneous lesions. In the absence of foreign body, infection, or another identifiable cause, fulfillment of both major and at least 1 minor criterion were proposed to establish the diagnosis of NXG.CONCLUSIONS AND RELEVAN...

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Section: Resultsmentioning

confidence: 89%

A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria

et al. 2020

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“…In over 90% of the patients, NXG involves more than one body site. Giant‐cell myocarditis should also be excluded …”

Section: Introductionmentioning

confidence: 99%

“…Management of NXG may be difficult with several treatment regimens including physical modalities, immunomodulatory drugs, immunosuppressive agents, high‐dose corticosteroids, alkylating agents, plasmapheresis and local radiotherapy showing variable dermatological responses …”

Section: Introductionmentioning

confidence: 99%

Treatment of necrobiotic xanthogranuloma – a systematic review

Miguel

Lukács

Illing

et al. 2016

Acad Dermatol Venereol

109

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Necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the periorbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. 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. The aim of this systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query 'necrobiotic xanthogranuloma treatment', both in English and German, about 'human' subjects and published between 1980 and 2014, documenting adequate treatment for NXG. Mainly individual case reports, small case series and retrospective studies were found. Treatment options include topical and systemic corticosteroids, thalidomide, high-dose intravenous immunoglobulin (IVIG), chlorambucil, cyclophosphamide, fludarabine, rituximab, melphalan, infliximab, interferon alpha, cladribine, hydroxychloroquine, azathioprine, methotrexate, laser therapy, radiotherapy, surgery, PUVA, plasmapheresis and extracorporeal photopheresis. Randomized controlled trials and studies on long-term outcomes after treatment were not found and are necessary to focus on in the future.

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“…NXG is categorized as non-Langerhans cell histiocytosis, often associated with monoclonal gammopathy (up to 90%). Some patients were reported to have multiple myeloma (10%), lymphoproliferative disorders, and/or atypical plasma cells in bone marrow examination [2, 3]. …”

Section: Discussionmentioning

confidence: 99%

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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Necrobiotic xanthogranuloma with multiple myeloma

Cited by 8 publications

References 4 publications

A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria

A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria

Treatment of necrobiotic xanthogranuloma – a systematic review

Necrobiotic Xanthogranuloma in a Patient with Multiple Myeloma

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