Necrobiotic xanthogranuloma successfully treated with a combination of dexamethasone and oral cyclophosphamide

Khan, Imran; Azam, Nasir; Sullivan, S. C.; Habboush, H.W.; Christian, Adam

doi:10.3129/i09-021

Cited by 8 publications

(7 citation statements)

References 4 publications

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“…First, HX and NX lesions can coexist with lesions of NXG in the same patient as seen both in patients from our series and from the literature. 46,[64][65][66][67] Second, the 3 forms share common histologic features, namely, the presence of foam cells and Touton cells. The latter is typically considered as characteristic of NXG; and, indeed, we did not find such cells in any of our patients with HX and NX.…”

Section: Discussionmentioning

confidence: 99%

“…In 5 patients, xanthoma and NXG were present at the same time. 46,[64][65][66][67] Extracutaneous lesions were reported in 23 cases (17.6%), including 6 patients with several localizations. They were pulmonary (n ϭ 7), 8 cardiac (n ϭ 6), 8,37 oral cavity (n ϭ 4), 38,65 orbital (n ϭ 3), 8 hepatic (n ϭ 2), 8,37 spleen (n ϭ 2), 8 medullar (n ϭ 1), 68 genital (penis; n ϭ 1), 34 mastoid (n ϭ 1), 32 facial nerve (n ϭ 1), 32 and renal (n ϭ 1).…”

Section: Comparison With Existing Literaturementioning

confidence: 99%

“…We reviewed 216 well-described cases of xanthomatosis associated with MIg in the literature, including 37 cases of HX, 3 63 of NX, 6,9-30 and 116 of NXG 4,8,[31][32][33][34][35][36][37][38][39][40][41][42][43][44][45][46][47] (Tables 3 and 4). The main patient characteristics are listed in Tables 3 and 4.…”

Section: Comparison With Existing Literaturementioning

confidence: 99%

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Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

Szalat

Arnulf

Karlin

et al. 2011

Blood

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Xanthomas are a common manifestation of lipid metabolism disorders. They include hyperlipemic xanthoma, normolipemic xanthoma, and a related condition, necrobiotic xanthogranuloma (NXG). All 3 forms can be associated with monoclonal immunoglobulin (MIg). In an attempt to improve diagnosis, understanding, and treatment of this association, we retrospectively analyzed a personal series of 24 patients (2 hyperlipemic xanthoma, 11 normolipemic xanthoma, and 11 NXG) and 230 well-documented reports from the literature. With the exception of the nodules and plaques featured in NXG, the clinical presentation of xanthomatous lesions usually resembled that seen in common hyperlipidemic forms and could not be used to suspect MIg-associated xanthomas. Extracutaneous sites were not rare. The MIg was an IgG in 80% of cases. Myeloma was diagnosed in 35%. Hypocomplementemia with low C4 fraction was present in 80% of studied patients. Low C1 inhibitor serum levels were found in 53%. Cryoglobulinemia was detected in

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Section: Discussionmentioning

confidence: 99%

Section: Comparison With Existing Literaturementioning

confidence: 99%

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Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

Szalat

Arnulf

Karlin

et al. 2011

Blood

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“…10 There are also several reports of effective treatment of NXG with chemotherapeutic agents particularly alkylating agents, such as chrorambucil, melphalan and CYC alone or in combination with corticosteroids. 5,6,8,11,12 Jakobiec suggests that patients without systemic disease receive a course of high-dose systemic steroids and periorbital radiotherapy; if the disease does not respond, then light chemotherapy with corticosteroids could be administered. 4 Long-term outcomes of PXAOA following various therapeutic modalities are rarely reported.…”

Section: Discussionmentioning

confidence: 99%

Periocular xanthogranuloma: A forgotten entity?

Papagoras

Kitsos²,

Zikou³

et al. 2010

OPTH

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Periocular xanthogranulomatous diseases are a rare group of disorders which are characterized by a predilection to affect the orbit and ocular adnexa and special histopathological features, in particular infiltrates comprising non-Langerhans-derived foamy histiocytes and Touton giant cells. The differential diagnosis is difficult and occasionally definite diagnosis cannot be established even after clinical and histopathological findings are taken together. We describe a case of a middle-aged man who presented with a 10-year history of voluminous eyelid swelling with concomitant late-onset atopic manifestations, namely bronchial asthma and allergic rhinitis with nasal polyps. After thorough clinical and laboratory investigation, including a biopsy of the eyelid, we classified the patient's disease to a rare entity that has been relatively recently described: periocular xanthogranuloma associated with adult-onset asthma. In a review of the literature, no prospective trials concerning the treatment of this disease were found. The literature mainly contained case reports and case series in which corticosteroids and chemotherapy with alkylating agents have been reported to be beneficial. We treated our patient with a combination of oral corticosteroids and cyclophosphamide pulses and we observed substantial regression of the eyelid masses together with a normalization of systemic immunologic abnormalities.

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“…Severe adverse events however limit the applicability of both drugs. Alternatively, oral cyclophosphamide (1 mg/kg per day for six months) can be used alone or in combination with steroids ( 112 , 115 , 116 ). Similarly, bortezomib alone or combined with steroids and/or lenalidomide/thalidomide is another choice for patients for whom chemotherapy is an option as it may lead to improved skin disease ( 66 , 67 ).…”

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 successfully treated with a combination of dexamethasone and oral cyclophosphamide

Cited by 8 publications

References 4 publications

Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

Periocular xanthogranuloma: A forgotten entity?

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

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