Premium
Pyoderma Gangrenosum Associated with Paroxysmal Nocturnal Hemoglobulinuria and Monoclonal Gammopathy
Author(s) -
Matsubara Kimiko,
Isoda Kenichi,
Maeda Yoshitami,
Mizutani Hitoshi
Publication year - 2002
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2002.tb00171.x
Subject(s) - paroxysmal nocturnal hemoglobinuria , pyoderma gangrenosum , immunology , cd59 , haematopoiesis , hemoglobinuria , pathogenesis , gammopathy , monoclonal , monoclonal antibody , medicine , biology , pathology , complement system , stem cell , antibody , hemolysis , microbiology and biotechnology , disease
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.