This report describes the case of a woman who was admitted to the hospital for highly destructive axial and peripheral arthropathy in association with acute malignant hypertension and skin purpura. Type I IgGK serum cryoglobulinemia was identified and was classified as a monoclonal gammopathy of unknown significance. Cryoglobulin was shown to crystallize in the serum and synovium fluid and was responsible for both granulomatous microcrystalline synovial inflammation and occlusive vasculopathy in the kidneys and skin. Cryocrystalglobulinemia pathogenicity and therapeutic implications are discussed.Monoclonal immunoglobulins may crystallize within or adjacent to plasma cells, mostly in multiple myeloma (1). Localized crystal deposits independent of plasma cells have been seen in various tissues with differing degrees of organ failure, including skin, cornea, synovium, testes, thyroid, adrenal glands, spleen, liver, lungs, coronary and renal arteries, renal glomeruli, and tubules. We report a case of cryocrystalglobulinemia responsible for skin and renal vasculopathy and associated with highly erosive arthropathy involving both peripheral and axial joints. CASE REPORTClinical course and laboratory findings. The patient, a 45-year-old woman, presented with severe polyarthropathy and gave a history of pain dating back to 1991. She had pain in multiple joints including the distal and proximal interphalangeal joints, metacarpophalangeal joints, wrists, elbows, shoulders, cervical spine, sacroiliac joints, heels, ankles, and metatarsophalangeal joints, which caused progressive disability. At different times on subsequent examinations there was gross and sometimes pseudotumoral swelling of the tendon sheaths of the fingers, dorsum of the hands, and elbows, and considerable restriction of movement of all involved joints. There was no rash on the palms or soles, acne, psoriasis, subcutaneous nodules, tophi, lymphadenopathy, or enlargement of the spleen or liver. Hemography results were normal.The erythrocyte sedimentation rate was 120 mmhour. The C-reactive protein level ranged from 2 mg/dl to 4 mg/dl. Liver test results and lactate dehydrogenase and muscle enzyme levels were within normal ranges. The uric acid level was 200 pMAiter. The serum calcium level was 2.5 M A i t e r , and the phosphate level was 1.5 M l l i t e r . Findings of serologic studies for infectious agents, including Lyme borreliosis, human immunodeficiency virus, parvovirus B19, and hepatitis B and C were negative. The results of tests for autoantibodies, including rheumatoid factor, antinuclear antibodies, anti-DNA, antiextractable nuclear antigen, antiphospholipid antibodies, antineutrophil cytoplasmic antibodies, and direct Coombs' test were negative. Serum C3 and C4 complement components and CH50 values were within normal ranges. HLA class I typing was negative for B27. A monoclonal cryoprecipitable 1gGK gammopa-
The most serious complication of Paget's disease is sarcomatous degeneration of pagetic bone. Multifocal sarcomatous degeneration occurs mainly in polyostotic Paget's disease. Multifocal Paget's sarcoma is uncommon and can arise in any site. We report two cases of synchronous multifocal sarcomatous degeneration. The two patients were elderly women (aged 77 and 86 years, respectively) who developed sarcomatous lesions concomitantly, in the first case report in left ilium, left tibia, and first lumbar vertebra and in the second case report in the skull, right ilium, and sacrum. Whether these cases are due to the simultaneous development of several primaries or to metastases from a single primary remains unclear.
This study described the imaging features of a distinctive pattern of biopsy-proven benign non-traumatic vertebral collapse (VC) that can mimic malignancy. Among 240 patients referred with painful VC over a 10-year period, we retrospectively selected 15 cases of benign VC which simulated malignancy, due to cortical bone destruction on radiographs. The diagnosis of benign origin was documented by percutaneous biopsy and 12-months of follow-up. Radiographs, CT and MR images of the spine were reviewed. Findings suggestive of malignancy included destruction involving the anterolateral cortex, posterior cortex and pedicles of the vertebral body (46%,15% and 15% respectively) at CT, epidural soft-tissue masses on CT (23%) and MR images (21%), and diffuse low signal-intensity (SI) of the vertebral body (50%) and pedicles (79%) on T1-weighted images. Features suggestive of a benign origin included an intravertebral vacuum phenomenon on radiographs and CT (13%), fracture lines within the vertebral body (92%) or pedicles (62%) at CT and presence an intravertebral area of high SI on T2-weighted images (93%). Benign non-traumatic osteolytic VC can simulate malignancy on radiographs. The features, above mentioned on CT and MR suggestive of a benign VC, are useful in interpreting biopsy results of such lesions and avoiding unnecessary repeat biopsy.
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