PMID- 2498140
OWN - NLM
STAT- MEDLINE
DCOM- 19890626
LR  - 20211203
IS  - 0011-9075 (Print)
IS  - 0011-9075 (Linking)
VI  - 178
IP  - 3
DP  - 1989
TI  - Studies on two siblings with recessive dystrophic epidermolysis bullosa 
      (Hallopeau-Siemens) and the plasminogen activator and its inhibitor in the 
      lesion.
PG  - 156-63
AB  - 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.
FAU - Kaneko, F
AU  - Kaneko F
AD  - Department of Dermatology, Hokkaido University School of Medicine, Japan.
FAU - Tsukinaga, I
AU  - Tsukinaga I
FAU - Ando, M
AU  - Ando M
FAU - Ohkawara, A
AU  - Ohkawara A
FAU - Oguchi, H
AU  - Oguchi H
FAU - Oikawa, K
AU  - Oikawa K
FAU - Nagai, M
AU  - Nagai M
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Switzerland
TA  - Dermatologica
JT  - Dermatologica
JID - 0211607
RN  - 0 (Esters)
RN  - 0 (Guanidines)
RN  - 0 (HLA Antigens)
RN  - 0 (Plasminogen Inactivators)
RN  - 0 (Protease Inhibitors)
RN  - 0FD207WKDU (camostat)
RN  - 4V7M9137X9 (Gabexate)
RN  - EC 3.4.21.- (Plasminogen Activators)
SB  - IM
MH  - Adolescent
MH  - Child
MH  - Epidermolysis Bullosa/*genetics/metabolism/pathology
MH  - Esters
MH  - Female
MH  - *Gabexate/*analogs & derivatives
MH  - Genes, Recessive
MH  - Guanidines/therapeutic use
MH  - HLA Antigens/analysis
MH  - Humans
MH  - Male
MH  - Pedigree
MH  - Plasminogen Activators/*analysis/antagonists & inhibitors
MH  - Plasminogen Inactivators
MH  - Protease Inhibitors/therapeutic use
EDAT- 1989/01/01 00:00
MHDA- 1989/01/01 00:01
CRDT- 1989/01/01 00:00
PHST- 1989/01/01 00:00 [pubmed]
PHST- 1989/01/01 00:01 [medline]
PHST- 1989/01/01 00:00 [entrez]
AID - 10.1159/000248416 [doi]
PST - ppublish
SO  - Dermatologica. 1989;178(3):156-63. doi: 10.1159/000248416.