Scleromyxedema is a rare and distinctive variant of cutaneous mucinoses of unknown etiology. It is presenting with generalized papular eruption and sclerodermoid induration. Numerous treatment modalities have been reported to produce partial or permanent responses. This study reports on a case of scleromyxedema without paraproteinemia in a subject who experienced a partial response to thalidomi...

Necrobiotic xanthogranuloma is a rare, idiopathic, and chronic systemic granulomatous disorder that is usually associated with paraproteinemia of the immunoglobulin (Ig)G kappa (κ) type. It is clinically characterized by yellowish red-to-brown indurated often ulcerating nodules or plaques that usually involve the periorbital regions of the elderly. We report an atypical case of NXG affecting on...

Skeletal-extraskeletal angiomatosis is defined as a benign vascular proliferation involving the medullary cavity of bone and at least one other type of tissue. It has also been known as cystic angiomatosis in which multiple cystic lesions are scattered diffusely throughout the skeleton often with similar angiomatous changes in other tissues, usually the spleen. A case of skeletal angiomatosis i...

Nonamyloidotic light-chain cardiomyopathy is a paraproteinemia-induced disorder. Unlike amyloidosis, light chain deposition may be reversible with appropriate treatment. We report a case of fatal light chain deposition disease manifested as cardiomyopathy, which was complicated by arterial embolism despite the maintenance of sinus rhythm.

Serum transient paraproteins are small monoclonal immunoglobulins that are induced by a self-limiting regulatory defect in the control of certain terminally differentiated B-cell clones. Relatively uncommon and ill-defined in the medical literature, such proteins may be regarded as immunologic anomalies. Their clinical significance and potential impact on patient management are poorly understoo...

M ONOCLONAL gammopathies are commonly associated with multiple myeloma and Waldenstr#{246}m's macroglobulinemia. These diseases generally occur only in older age groups. Paraproteinemias have also been oh-served in myeloproliferative disorders,1 as well as in association with lympho-mas.2 However, paraproteinemia in children is an extremely rare condition, and only very few cases have been reco...