Pyoderma Gangrenosum Associated with Paroxysmal Nocturnal Hemoglobulinuria and Monoclonal Gammopathy.
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Accession number;02A0215986
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| Title;Pyoderma Gangrenosum Associated with Paroxysmal Nocturnal Hemoglobulinuria and Monoclonal Gammopathy. |
| Author;
MATSUBARA K
(Mie Univ., Tsu, Jpn)
ISODA K
(Mie Univ., Tsu, Jpn)
MAEDA Y
(Mie Univ., Tsu, Jpn)
MIZUTANI H
(Mie Univ., Tsu, Jpn)
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Journal Title;J Dermatol
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Journal Code:Z0757A
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ISSN:0385-2407
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VOL.29;NO.2;PAGE.86-90(2002)
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| Figure&Table&Reference;FIG.2, REF.11 |
| Pub. Country;Japan |
| Language;English |
| Abstract;Pyoderma gangrenosum developed in a man with a five-year history of paroxysmal nocturnal hemoglobinuria and monoclonal gammopathy. He had multiple walnut sized ulcers on his back and extremities, plasma IgM-k type M-protein and low erythrocytic CD55 expression. This is an extremely rare association. However, clonal expansion of plasma cells and chimeric expression of hematopoietic cell glycosylphosphatidylinositol (GPI)-anchored proteins may represent somatic mutations of hematopoietic stem cells in PG as well as PNH. PNH is based on abnormalities in the GPI-anchor formation on various hematopoietic and non-hematopoietic cells. Since the GPI-anchored proteins have pleiotropic functions in complement mediated cell lysis, leukocyte motility, and coagulation systems, the present case may indicate the possible involvement of a GPI-anchored protein abnormality in the pathogenesis of PG. (author abst.) |
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