Complete Response to Thalidomide and Dexamethasone in a Patient With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathy: A Case Report and Review of the Literature

Efebera, Yvonne A.; Blanchard, Elizabeth; Allam, Charles; Han, Andrew; Lee, Saem; Munshi, Nikhil C.

doi:10.1016/j.clml.2011.03.020

Cited by 30 publications

(17 citation statements)

References 25 publications

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“…Treatment modalities include surgical excision, plasmapheresis, systemic and intralesional glucocorticoids, alkylating agents including chlorambucil and melphalan, antimetabolites (methotrexate, azathioprine), and antimicrobials (dapsone, clofazamine) . Chemotherapy regimens including lenalidomide and dexamethasone have been used in NXG patients with associated monoclonal gammopathy . One report described the successful use of intravenous immunoglobulin with minimal side effects .…”

Section: Discussionmentioning

confidence: 99%

A 55‐Year‐Old Man With Periorbital and Inguinal Masses, Pericarditis, and Pleuritis

Gupta

Frater

et al. 2017

Arthritis Care & Research

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A 55-year-old male was referred to the rheumatology clinic for evaluation of recurrent inguinal and periorbital masses. History of the present illnessA 55-year-old white man with ulcerative colitis in remission and coronary artery disease developed an indurated 5-cm right inguinal subcutaneous mass approximately 6 years prior to presentation. An excisional biopsy demonstrated lymphoid follicular hyperplasia. Three years later, the right inguinal mass reappeared. Repeat surgical excision revealed a xanthogranulomatous infiltrate with necrobiosis, cholesterol clefts, and Touton giant cells (large multinucleated cells with a ring of nuclei surrounded by foamy cytoplasm). Gomori methenamine silver and Fite stains were negative for fungal or mycobacterial organisms. Despite removal, the mass reappeared within 4 months and slowly but progressively increased in size, accompanied by hyperpigmentation and a yellow discoloration of the overlying skin. One year prior to presentation, he developed shortness of breath and was found to have pericarditis complicated by pericardial effusion and tamponade for which he underwent a pericardiocentesis and pericardial window. Cytology of the fluid was negative for malignant cells. A pericardial biopsy demonstrated a thickened pericardial wall with florid chronic inflammation, fibrosis, and numerous plasma cells. In situ hybridization studies showed a kappa restricted monoclonal plasma cell population. Around that time, he also developed a tight band-like mass at the base of his penis. The right inguinal mass continued to increase in size, and ulceration of the overlying skin developed. Nine months prior to presentation, he noted a left periorbital 1-cm mass that increased in size, leading to ptosis and decreased peripheral vision. He also developed a right periorbital mass that was smaller in size. Computed tomography (CT) of the orbits showed bilateral lacrimal gland enlargement. Biopsy findings of the left periorbital mass showed a lymphoplasmacytic infiltrate with intervening areas of fibrosis, focal areas of necrobiosis, and Touton giant cells. Flow cytometry of this tissue showed a polyclonal B cell population. Two months prior to presentation, he developed shortness of breath and fatigue, and a chest radiograph demonstrated bilateral pleural effusions. The pleural fluid had a pH of 7.29. There were 164,000 red blood cells and 1,952 nucleated cells with a differential of 29% neutrophils, 52% lymphocytes, 7% monocytes, and 12% eosinophils. The glucose was less than 20 mg/dl. The protein was 5.7 gm/dl, and lactate dehydrogenase was 2,474 units/liter. Cultures and cytology findings were negative. Around the same time, he developed an erythematous, maculopapular rash on the lower extremities that resolved spontaneously in a week. He presented 2 months later to the rheumatology clinic for further evaluation. Past medical history and medicationsHe had a history of ulcerative colitis diagnosed at age 27 years, which was in remission on mesalamine, hyperlipidemia (lipid profile at age 5...

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Section: Discussionmentioning

confidence: 99%

A 55‐Year‐Old Man With Periorbital and Inguinal Masses, Pericarditis, and Pleuritis

Gupta

Frater

et al. 2017

Arthritis Care & Research

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“…No first-line therapy has been established for NXG. The recommended therapies of intralesional and/or systemic corticosteroids (1,2), alkylating agents (such as melphalan (3), chlorambucil (4), or cyclophosphamide (5)), lenalidomide (6), thalidomide (7), and intravenous immunoglobulin (2) have all shown inconsistent success. (hematoxylin and eosin stain, ×200).…”

Section: Discussionmentioning

confidence: 99%

Necrobiotic xanthogranuloma: response to dapsone

Wei

Cheng

et al. 2014

Dermatol Ther

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“…2 Other sites of involvement include the face, trunk, and extremities. 3,4 There are only 2 cases of NXG with mucosal or genital involvement reported in the literature. Fortson and Schroeter 5 reported a case of NXG of the lips and tongue, and a review by Wood et al 1 cited a 26-year-old man with penile NXG in a table summarizing the clinical findings in 12 patients with NXG.…”

Section: Vulvar Necrobiotic Xanthogranulomamentioning

confidence: 97%