We report a case of urticarial vasculitis associated with macroglobulinemia in a 50-year-old woman. She suffered from intermittent fever with wheals persisting for more than 24 hours. Skin histology revealed perivascular neutrophilic infiltrates with nuclear dusts. Laboratory study demonstrated leucocytosis and a high serum IgM level (2350 mg/dl) with an M component. No overt lymphoid proliferative disease was found. As far as we know, this is the first report of such a case in Japan. Predonizolone and nonsteroidal anti-inflammatory drugs have proved transiently effective for controlling the urticaria, but not for the IgM gammopathy.
A novel metabolic disease, angiokeratoma corporis diffusum (Kanzaki), was the subject of an extensive histopathologic and ultrastructural study. Findings included dilated lymph and blood vessels in the upper dermis with an orthokeratortic, thickened, horny layer in well developed angiokeratoma. In the early papules, a few sporadic dyskeratotic keratinocytes were present in the epidermis with or without a thickened horny layer. Vesicular clear vacuolation was clearly observed in the cytoplasm of the secretory portion of the eccrine sweat glands, but none was observed in the vascular endothelial cells with hematoxylin-eosin staining. Using electron microscopy, lysosomal vacuolation was observed in many cell types, including eccrine sweat gland cells, vascular endothelial cells, dermal fibroblasts, dermal neural cells, lymphocytes of peripheral blood in the skin, and glomerular endothelial cells, but none was noted in the epithelial cells of the kidney. Widely dilated vacuoles were found to contain only a small amount of fuzzy filamentous material in the vascular endothelial cells, filamentous or electron-dense granular substances in fibroblasts, and electron-dense, lamellated or homogeneous structures in eccrine sweat gland cells and in neural cells. Ultracytochemical examination revealed glycoconjugates in dilated lysosomes. Characteristics of Kanzaki Disease were shown to differ from those of Fabry disease or any other lysosomal storage disease.
Muir-Torre syndrome is characterized by multiple sebaceous tumors, various internal malignancies and an autosomal dominant inheritance. We herein report a typical case. The patient was a 69-year-old man with sebaceous adenomas, a keratoacanthoma, and actinic keratosis in addition to carcinomas of the prostate, colon, duodenum, and larynx. His family members also suffered from multiple cancers.
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