Necrobiotic xanthogranuloma. Differential diagnosis, treatment and systemic involvement. Case report

Sanz-Marco, E.; España, Enrique; López-Prats, M.J.; Chirivella-Casanova, M.; Aviño, J.; Díaz-Llopis, M.

doi:10.1016/j.oftale.2014.07.005

Cited by 4 publications

(4 citation statements)

References 5 publications

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“…Histology in NXG is characterized by collagen necrobiosis, Touton giant cells, cholesterol clefts, lymphoid aggregates, and foamy histiocytes that extend from the dermis into the subcutaneous tissue . Many patients have an associated monoclonal gammopathy, most often of the IgG‐kappa type . Other disease associations include multiple myeloma, lymphoma, and chronic lymphocytic leukemia .…”

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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“…He also experienced paroxysmal loss of consciousness and limb convulsions for 2 years, and was on oral levetiracetam tablets for antiepileptic treatment, with no current epilepsy symptoms. Laboratory biochemical tests revealed the following: an erythrocyte sedimentation rate (ESR) of 120 mm/h (normal range, 0-20 mm/h), a white blood cell count (WBC) of 11.53×10 9 /L [normal range, (3.5-9.5)×10 9 /L], a high-sensitivity C-reactive protein (hsCRP) level of 84.6 (normal range, 0-7.44), a highdensity lipoprotein cholesterol (HDL-C) level of 0.82 mmol/L (normal range, 0.9-2 mmol/L), an IgG (λ) M-protein-positive status in serum protein electrophoresis, and an IgG level of 33.30 g/L (normal range, 7.82-16.8 g/L). The lambda light chain was 31.8 g/L (normal range, 3.13-7.23 g/L).…”

Section: Case Presentationmentioning

confidence: 99%

An unusual case of necrobiotic xanthogranuloma with IgG-λ monoclonal gammopathy presenting as cerebral manifestations

Li¹,

Chen²,

Cheng³

et al. 2023

Quant Imaging Med Surg

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“…Hence, in addition to addressing treatment of orbital NBX manifestations, patients in whom NBX has been diagnosed or suspected should undergo investigation up to rule out hematological malignancies. Similar to AOX and AAPOX, NBX has also been treated with systemic and intralesional steroids in the literature, often with recurrence following taper [37,40,45]. Cyclosporine, chlorambucil, and melphalan have also each successfully been used anecdotally in treatment of NBX [45][46][47].…”

Section: Aox Adult Aapx Nbxmentioning

confidence: 99%

“…Similar to AOX and AAPOX, NBX has also been treated with systemic and intralesional steroids in the literature, often with recurrence following taper [37,40,45]. Cyclosporine, chlorambucil, and melphalan have also each successfully been used anecdotally in treatment of NBX [45][46][47]. Regarding surgical excision of NBX lesions, Ugurlu et al reported that lesions recurred in 42% of patients following surgery and hence recommended against it [44].…”

Section: Aox Adult Aapx Nbxmentioning

confidence: 99%

Orbital inflammatory disease management

Moore

Rootman

2016

Expert Review of Ophthalmology

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Necrobiotic xanthogranuloma. Differential diagnosis, treatment and systemic involvement. Case report

Cited by 4 publications

References 5 publications

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

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

An unusual case of necrobiotic xanthogranuloma with IgG-λ monoclonal gammopathy presenting as cerebral manifestations

Orbital inflammatory disease management

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