title: Studies on two siblings with recessive dystrophic epidermolysis bullosa (Hallopeau-Siemens) and the plasminogen activator and its inhibitor in the lesion.
abstract: Activities of plasminogen activators (PA) and their inhibitors were studied in the bullous lesions of 2 siblings with recessive dystrophic epidermolysis bullosa (RDEB). The hematological findings of the patients revealed hyperfibrinogenemia, hyper-gamma-globulinemia and marked thrombocytosis. The immunofluorescent studies showed strong deposits of plasminogen, fibrin-degenerative products and alpha 1-antitrypsin around the blister lesions. On the other hand, alpha 2-macroglobulin (alpha 2-M) was sparsely deposited. The levels of alpha 2-M in the blister fluid of the patients were also decreased in comparison with those from patients with other bullous dermatoses. The topical application of antibiotic ointment with strong PA inhibitor was clinically effective when applied to the blister and eroded lesions of the patients. These findings suggest that the increased activity of PA may play an important role in the development of blister formation in patients with RDEB.
authors.affiliation: Department of Dermatology, Hokkaido University School of Medicine, Japan.
authors.forename: F
authors.initials: F
authors.lastname: Kaneko
authors.forename: I
authors.initials: I
authors.lastname: Tsukinaga
authors.forename: M
authors.initials: M
authors.lastname: Ando
authors.forename: A
authors.initials: A
authors.lastname: Ohkawara
authors.forename: H
authors.initials: H
authors.lastname: Oguchi
authors.forename: K
authors.initials: K
authors.lastname: Oikawa
authors.forename: M
authors.initials: M
authors.lastname: Nagai
mesh_terms: D000293:Adolescent; D002648:Child; D004820:Epidermolysis Bullosa / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D004952:Esters; D005260:Female; D016670:Gabexate / Q000031:analogs & derivatives*; D005808:Genes, Recessive; D006146:Guanidines / Q000627:therapeutic use; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D008297:Male; D010375:Pedigree; D010960:Plasminogen Activators / Q000032:analysis* / Q000037:antagonists & inhibitors; D015849:Plasminogen Inactivators; D011480:Protease Inhibitors / Q000627:therapeutic use
chemical_list: D004952:Esters; D006146:Guanidines; D006680:HLA Antigens; D015849:Plasminogen Inactivators; D011480:Protease Inhibitors; C034532:camostat; D016670:Gabexate; D010960:Plasminogen Activators