Pyoderma gangrenosum associated with paroxysmal nocturnal hemoglobulinuria and monoclonal gammopathy.
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.
Matsubara
Kimiko
K
Department of Dermatology, Mie University, Faculty of Medicine, Tsu, Japan.
Isoda
Ken-ichi
K
Maeda
Yoshitami
Y
Mizutani
Hitoshi
H
D001707:Biopsy, Needle; D005500:Follow-Up Studies; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D011239:Prednisolone / Q000008:administration & dosage; D017511:Pyoderma Gangrenosum / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D012720:Severity of Illness Index
D011239:Prednisolone