Necrobiosis and T-lymphocyte infiltration in retrieved aseptically loosened metal-on-polyethylene arthroplasties.

BACKGROUND AND PURPOSE: Soft tissue necrobiosis and T-lymphocyte infiltration within the periprosthetic soft tissue have been linked to a suggested hypersensitivity reaction of the delayed-type following the metal-on-metal arthroplasty. While we observed both synovial necrobiosis and lymphocyte infiltrates in synovial tissues with failed metal-on-polyethylene prostheses, we hypothesized that both findings are unspecific for metal-on-metal bearing coupes. Thus, we wished to quantify the extent of necrobiosis and the amount of T-lymphocyte infiltration in aseptically loosened metal-on-polyethylene prostheses. MATERIALS AND METHODS: We analyzed 28 consecutive synovial biopsy specimens obtained at revision surgery of aseptically loosened metal-on-polyethylene prostheses (19 hips and 9 knees) and quantified both the extent of necrobiosis vertically and the density of CD3+, CD4+, and CD8+ lymphocytes within the joint capsular tissue. We excluded patients with inflammatory skeletal disease or with a history of metal hypersensitivity. RESULTS: We found necrobiosis in 23 of 28 cases and it was most often connected with the superficial portions of the synovium. Necrobiosis of deeper tissues was seen in 8 specimens and it was strongly associated with superficial necrobiosis. While CD3+ lymphocytes were detected in each biopsy, 4 cases with more than 300 CD3+ lymphocytes were identified in the group of 26 cases that presented with more than 100 CD3+ lymphocytes within one high-power field. 16 cases with more than 100 CD3+ lymphocytes also showed concomitant superficial necrobiosis of the synovium. In the inflammatory infiltration of periprosthetic synovium, CD8+ lymphocytes predominated over CD4+ cells. INTERPRETATION: Synovial necrobiosis and infiltration of T-lymphocytes are common findings in tissues around aseptically loosened metal-on-polyethylene arthroplasty in patients without a clinically suspected metal hypersensitivity reaction. Thus, neither necrobiosis nor infiltration of T-lymphocytes should be considered to be specific for failed metal-on-metal bearings or metal hypersensitivity reaction.

Necrobiotic xanthogranuloma-rapid progression under treatment with melphalan.

Necrobiotic xanthogranuloma is a systemic disease associated in most cases with monoclonal paraproteinaemia. Although the causative role of the paraproteinaemia is supposed, the pathogenesis of this disease remains unknown. We report a 65-year-old woman with a 28-year history of disseminated indurated plaques involving her face, trunk and extremities. Since 1994 an IgG kappa paraproteinaemia was present. Current biopsies showed typical histological features of necrobiotic xanthogranuloma. We treated this extraordinary widespread condition with melphalan and prednisolone. Although the serum levels of IgG kappa light chains and gamma globulins decreased, the cutaneous plaques extended rapidly in size and number, which casts doubt on the causative significance of the paraproteinaemia in the pathogenesis of necrobiotic xanthogranuloma. The paraproteinaemia in patients with necrobiotic xanthogranuloma may rather reflect a secondary phenomenon than the originating cause. Pathogenetic and therapeutical concepts based thereon that have been proposed so far should be critically reconsidered.

[Systemic necrobiotic xanthogranuloma with initial pericardial and pulmonary involvement]. / Xanthogranulome nécrobiotique révélé par une atteinte péricardique et pulmonaire.

INTRODUCTION: Necrobiotic xanthogranuloma is a rare disease, usually associated with a monoclonal gammapathy. We report a case with pericardial and pulmonary involvement that preceded the appearance of the cutaneous lesions. OBSERVATION: A 58 year-old woman was hospitalized for cardiac tamponade. She has a past history of a ductal carcinoma of the right breast that had been completely cured. Pericardial and pulmonary samples showed a predominantly histiocyte monomorphous infiltrate. Twelve months later, indurated papular-like cutaneous lesions appeared around the eye orbits and on the thorax. The histological examination of the skin revealed a necrobiotic xanthogranuloma and the diagnosis of cutaneous and visceral necrobiotic xanthogranuloma was retained. The patient exhibited an IgG Kappa monoclonal gammapathy of undetermined significance. Sequential treatment with melphalan and general corticosteroids moderately improved the cutaneous lesions but did not modify the monoclonal peak. DISCUSSION: To our knowledge, this is the first case of pericardial involvement of a necrobiotic xanthogranuloma. The discovery of the disease, revealed by a visceral manifestation at the onset is uncommon. The subsequent appearance of typical cutaneous lesions permits the retrospective diagnosis of systemic necrobiotic xanthogranuloma. Because its diagnosis is difficult in the absence of concomitant cutaneous involvement, visceral localizations of the disease must be recognized.

Unusual clinical manifestation of a disfiguring necrobiotic granulomatous disease.

A 39-year-old woman had a disfiguring granulomatous disease of 36 years' duration. Nodules appeared on her lower extremities and later extended to her face; this led to ulceration and destruction of the nasal cartilage. This disease of unknown origin does not conform to any previously described entity. Some patients with a similar case presentation have been observed in France.

[Necrobiotic xanthogranuloma in IgG kappa plasmacytoma and Quincke edema]. / Nekrobiotisches Xanthogranulom bei IgG-kappa-Plasmozytom und Quincke-Odem.

Necrobiotic xanthogranuloma is a non-X histiocytosis with unknown pathogenesis. It is associated with paraproteinaemia, and in rare cases with multiple myeloma. A decreased level of C1 inhibitor has been found in several cases without clinical manifestations of Quincke oedema. We report on a patient with necrobiotic xanthogranuloma and myeloma, in whom we found a decreased level of C1 inhibitor and recurrent episodes of manifest Quincke oedema. The indirect detection of auto-antibodies against the paraprotein with development of immune complexes is regarded as an explanation for the consumption of the C1 inactivator and the manifestation of Quincke oedema. The possibility of a causal relationship between paraproteinaemia and necrotic xanthogranuloma is discussed.

Necrobiotic xanthogranuloma in a human T-lymphotropic virus type 1 carrier.

A 67-year-old man who had necrobiotic xanthogranuloma associated with paraproteinemia is described. He was a human T-lymphotropic virus type 1 (HTLV-1) carrier who had a high titer of circulating anti-HTLV-1 antibodies and neurologic abnormalities that suggested HTLV-1-associated myelopathy. The patient's necrobiotic xanthogranuloma and neurologic symptoms improved after he received four 5-day courses of melphalan, 2 mg/day, and prednisolone, 20 mg/day, at 4-week intervals.