[Capillary leak syndrome disclosing Ofuji's papuloerythroderma].
BACKGROUND
Capillary leak syndrome is a specific entity among syndromes with capillary hyperpermeability. Endothelial cell activation is related to the higt level of adhesion molecules (sICAM-1, sVCAM-&, sCD62E) possibly due to several cytokines (IL-2, TNF ...).


METHODS
An 84-year-old woman was hospitalized for erythroderma. Ofujui papuloerythroderma was diagnosed and edema was attributed to capillary leak. A kinetic study of several cytokines and adhesion molecules sCD62E, sVCAM-1 and sICAM-1 was done. Outcome was favorable with corticopuvatherapy.


CONCLUSIONS
The capillary leak syndrome reported here is simlar to that described in other erythrodermas with or without lymphoma. The keratinocyte would be activated by the CD4 T lymphocyte via the gamma-interferon mediator. The T cell secretes cytokines (interleukin-1, tumor necrosis factor ...) which activates the endothelium and increases vascular permeability. The level of adhesion molecules and changes observed during the episode of edema demonstrated the endothelial activation.
Carsuzaa
F
F
Service de Dermatologie, HIA Sainte-Anne, Toulon Naval.
Pierre
C
C
Morand
J J
JJ
Farnarier
C
C
Marrot
F
F
Kaplanski
G
G
D000368:Aged; D000369:Aged, 80 and over; D019559:Capillary Leak Syndrome / Q000209:etiology* / Q000503:physiopathology; D015815:Cell Adhesion Molecules / Q000032:analysis; D016207:Cytokines / Q000032:analysis; D003873:Dermatitis, Exfoliative / Q000150:complications* / Q000175:diagnosis; D004727:Endothelium / Q000378:metabolism; D005260:Female; D006801:Humans; D007150:Immunohistochemistry
D015815:Cell Adhesion Molecules; D016207:Cytokines
Syndrome de fuite capillaire révélant une papulo-erythrodermie d'Ofuji.