Necrobiotic xanthogranuloma: response to dapsone

Wei, Yu-Hsiang; Cheng, Jen-Jung; Wu, Yung-Chun; Liu, Chin-Yin; Hung, Chu-Ju; Hsu, Jeng-Dong; Hsiao, Yu‐Ping

doi:10.1111/dth.12179

Cited by 9 publications

(3 citation statements)

References 9 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: 99%

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: 99%

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

et al. 2020

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“…Therapeutic modalities for NXG have been highly variable and very often unsuccessful. Previously reported pharmacotherapies include intralesional and systemic glucocorticoids, high‐dose intravenous immunoglobulin, antimetabolites, and alkylating agents such as chlorambucil, cyclophosphamide, and melphalan . Response to therapy, although not standardized in the published reports, seems to depend on whether there is an associated hematologic malignancy.…”

Section: Discussionmentioning

confidence: 99%

Hypercalcemia in Necrobiotic Xanthogranuloma: First Reported Case and Insight Into Treatment

Sfeir

Zogala

Popii

2016

Journal of Bone and Mineral Research

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Necrobiotic xanthogranuloma (NXG) is a rare systemic and progressive granulomatous disease first described in 1980. Given no established first-line therapy, treatment focuses on the control of skin lesions and associated complications. Despite it being a granulomatous disease, NXG has not been associated with hypercalcemia. About 140 cases of NXG have been reported to date but, to our knowledge, this is the first case to be complicated by hypercalcemia. Our case confirms a granulomatous disease-mediated production of 1α-hydroxylase leading to increased synthesis of 1,25-dihydroxyvitamin D and subsequent hypercalcemia. Based on this pathophysiology, we elected to start systemic glucocorticoids, titrated to clinical and metabolic response. Steroid-sparing agents need to be considered to avoid long-term complications but continue controlling this granulomatous disease. © 2017 American Society for Bone and Mineral Research.

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“…Surgery is an important component of the management of these patients both from cosmetic and functional point of view ( 126 , 127 ). Other potential therapy options may include dapsone ( 128 ), antimalarials ( 111 ), and biologics ( 125 , 129 , 130 ).…”

Section: Mgus-associated Cutaneous Involvementmentioning

confidence: 99%

Monoclonal gammopathies of clinical significance (MGCS): In pursuit of optimal treatment

et al. 2022

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Monoclonal gammopathy of clinical significance (MGCS) represents a new clinical entity referring to a myriad of pathological conditions associated with the monoclonal gammopathy of undetermined significance (MGUS). The establishment of MGCS expands our current understanding of the pathophysiology of a range of diseases, in which the M protein is often found. Aside from the kidney, the three main organ systems most affected by monoclonal gammopathy include the peripheral nervous system, skin, and eye. The optimal management of these MGUS-related conditions is not known yet due to the paucity of clinical data, the rarity of some syndromes, and limited awareness among healthcare professionals. Currently, two main treatment approaches exist. The first one resembles the now-established therapeutic strategy for monoclonal gammopathy of renal significance (MGRS), in which chemotherapy with anti-myeloma agents is used to target clonal lesion that is thought to be the culprit of the complex clinical presentation. The second approach includes various systemic immunomodulatory or immunosuppressive options, including intravenous immunoglobulins, corticosteroids, or biological agents. Although some conditions of the MGCS spectrum can be effectively managed with therapies aiming at the etiology or pathogenesis of the disease, evidence regarding other pathologies is severely limited to individual patient data from case reports or series. Future research should pursue filling the gap in knowledge and finding the optimal treatment for this novel clinical category.

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Necrobiotic xanthogranuloma: response to dapsone

Cited by 9 publications

References 9 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

Hypercalcemia in Necrobiotic Xanthogranuloma: First Reported Case and Insight Into Treatment

Monoclonal gammopathies of clinical significance (MGCS): In pursuit of optimal treatment

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