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

Abstract

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...

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

“…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).…”

Pleural effusion related to IgG4

“…Therefore, relying on IgG4 levels alone may result in under-diagnosis or over-diagnosis, and the lack of elevated IgG4 level does not exclude this diagnosis. Inflammatory markers such as C-reactive protein and LDH levels are often within normal limits in IgG4-RD [30 ▪▪ ,35] although LDH levels were elevated in some cases [25 ▪ ,36]. In general, IgG4-RD tends to show relatively weak inflammation signs [37].…”