A 62-year-old woman had multiple plasmacytomas in the skin and lymph nodes, without Bence-Jones protein or a monoclonal peak of serum immunoglobulins. Infiltrating plasmacytoid cells expressed cytoplasmic IgG (lambda) and surface CD38, without any B-cell markers. There was no visceral or bone marrow involvement suggestive of multiple myeloma. Southern blot analysis of extracted DNA from the cutaneous lesions showed two rearranged bands with an immunoglobulin, but not a T-cell receptor, gene probe. The patient showed a poor response to chemotherapy, and died of bronchopneumonia. The clinical course and cytological features differentiate multiple cutaneous extramedullary plasmacytomas from solitary cutaneous extramedullary plasmacytoma and cutaneous lesions associated with multiple myeloma.
We have encountered 2 cases (mother and son) with an autosomal dominant form of cutis laxa, which is clinically characterized by inelastic, loose, and pendulous skin without systemic organ involvement. The histopathological findings disclosed that the elastic fibers were decreased in number and had abnormalities in shape. Furthermore, electron microscopic observations showed some variations in structural abnormalities of the elastic fibers between the 2 cases.These findings may suggest that the elastic fibers seen in congenital cutis laxa give various features for elastogenesis and elastolysis in the individual cases, perhaps due to aging.
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