Osteosclerotic Bone Lesions Research Articles

A Case of Systemic Osteomyelitides Due to Mycobacterium avium

A case of multiple osteomyelitides due to Mycobacterium avium (M. avium) infection with osteosclerotic bone lesions is reported. A 67-year-old male had been suffering from persistent fever and back pain since October 1999, and 20.0-2.5 mg prednisolone per day was prescribed for continuous inflammatory symptoms in January 2000. Six months later, computed tomography revealed osteosclerotic lesions in the left femur and thoracic vertebrae, but no skin lesion associated with mastocytosis or internal malignancy was identified. In September of 2002, a dome-shaped, soft subcutaneous tumor developed on the upper sternum. Histopathological findings revealed subcutaneous adipose tissue with several foci of tiny abscesses. Two weeks later, creamy pus was discharged through a draining sinus at the center of the wound. M. avium was demonstrated in the pus by Zeel-Nielsen staining and microplate hybridization.

Schnitzler syndrome: heterogeneous immunopathological findings involving IgM-skin interactions.

The Schnitzler syndrome is the association of chronic urticaria, intermittent fever, osteosclerotic bone lesions and a monoclonal IgM gammopathy. It is not yet firmly established whether the monoclonal immunoglobulin component plays a part in the pathophysiology of the urticarial lesions. Immunoblotting on epidermal and dermal human skin extracts as well as immunoelectron microscopic (IEM) studies on Lowicryl K4M-embedded skin sections were performed in three patients with the Schnitzler syndrome. Western blotting on epidermal extracts showed the presence of IgM-kappa antiskin autoantibodies in two patients. These antibodies displayed the same isotype as the monoclonal components and recognized a 280-290-kDa antigen in one patient and a 200-kDa antigen in the other patient. IEM studies showed sparse IgM deposits in the epidermis, around the keratinocytes, near the desmosomes in one patient and dense deposits below the lamina densa, in the region of the anchoring fibrils, in another patient. Antiskin IgM autoantibodies of the same isotype as their monoclonal gammopathies can be present in the serum of some patients with the Schnitzler syndrome. These IgM antibodies seem to deposit in vivo in the epidermis and at the dermal-epidermal junction, in the region of the anchoring fibrils. These findings suggest that the monoclonal gammopathy plays a part in the pathophysiology of the skin rash. They also suggest patient heterogeneity both in the skin antigens that are recognized as well as in their localization.

Dense bone around the sacroiliac joint: a radiological review of the differential diagnosis

Solitary osteosclerotic bone lesions around the sacroiliac (SI) joints are described as seen on plain radiographs or conventional tomography, with the descriptive attributes ranging from round to ovoid over square and triangular to multiform. The etiology of the solitary osteosclerotic lesions around the SI joint can often be concluded from the radiographic appearance, sometimes complemented by the presentation on CT and/or scintigraphy.

POEMS syndrome.

The principal features of POEMS syndrome are osteosclerotic bone lesions, peripheral sensorimotor neuropathy, and elevated levels of a monoclonal protein in the serum or urine. Skin lesions are present in the majority of patients with POEMS syndrome. Diffuse hyperpigmentation is the most common cutaneous finding. Hemangiomas with histopathological features resembling renal glomeruli are the most specific skin lesions.

Osteopoikilosis and the Buschke–Ollendorff syndrome

Osteopoikilosis is an uncommon cause of multiple osteosclerotic bone lesions. The skeletal changes of osteopoikilosis (synonyms: osteopathia condensans disseminata, spotted bone) were first described by Stieda in 1905 and subsequently by Albers-Schoenberg (1915). Radiologically, the lesions consist of multiple wellcircumscribed round or oval opacities, each 1–10 mm in diameter. The mottling is more marked in the epiphyses and metaphyses of the long bones and the pelvis, but is also common in the spongiosa of the phalanges, carpal and tarsal bones. The changes are more common in men than women and usually present in adulthood, although osteopoikilosis has been reported in children. Familial aggregation has been described, and autosomal dominant inheritance with variable expression is proposed (Schoenenberg, 1975). Osteopoikilosis should be considered in the differential diagnosis of multiple osteosclerotic bone lesions, but is a relatively uncommon finding. Serowy (1956) could find only 100 reported cases in the literature over a 25-year period since its initial description, and Jonasch (1955) found only 12 cases of osteopoikilosis in a series of 21000 radiographs.

Plasma-cell dyscrasia with polyneuropathy. The spectrum of POEMS syndrome.

The POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome and osteosclerotic myeloma (polyneuropathy and sclerotic bone lesions) may both be manifestations of plasma-cell dyscrasia, but the interrelation of these diseases is not clear. We therefore set out to define the clinical spectrum of disease in patients with plasma-cell dyscrasia and polyneuropathy who have the complete or incomplete form of the POEMS syndrome or osteosclerotic myeloma. Among 2714 patients with plasma-cell dyscrasia who were identified between 1973 and 1989, we reviewed the cases of those with polyneuropathy and plasma-cell dyscrasia who fulfilled the criteria for the POEMS syndrome or osteosclerotic myeloma. Thirty-eight patients (1.4 percent) with a median age of 51 years were identified, 22 of whom were male. By definition, all had polyneuropathy (37 combined sensorimotor, and 1 primarily motor). Other findings included osteosclerotic bone lesions (82 percent), skin abnormalities (58 percent), lymphadenopathy (42 percent), papilledema (37 percent), peripheral edema (29 percent), hepatomegaly (24 percent), splenomegaly (21 percent), and ascites (11 percent). Thirty-three patients (87 percent) had an abnormal M protein in serum or urine (17 had IgA lambda, and 12 IgG lambda). Five patients fulfilled all the criteria for the POEMS syndrome. The estimated five-year survival in the 38 patients was 60 percent, which was significantly better than the 20 percent survival in 869 patients with multiple myeloma (P < 0.05). The clinical course was similar among the patients with the complete form of the POEMS syndrome and those with the incomplete form. Plasma-cell dyscrasia with polyneuropathy is a rare multisystem disease that often presents with osteosclerotic bone lesions. The differentiation of the POEMS syndrome from so-called osteosclerotic myeloma with peripheral neuropathy appears to have no clinical value.

Osteosclerotic myeloma with polyneuropathy and hypocalcemia

A case is presented of a 46-year-old man with multifocal osteosclerotic bone lesions, peripheral polyneuropathy and hypocalcemia. Histologic examination of a bone marrow biopsy disclosed a multiple myeloma. Immunoelectrophoresis revealed a small M-component identified as IgG-lambda. Osteosclerotic myeloma lacking any osteolytic lesions seems to be very rare and shows several different features as compared with classical myeloma. A review of the current literature suggests that multiple myeloma is not a uniform disease but rather a group of clinical syndromes characterized by the special properties of their proliferating plasma cell clones.