Denes E, Devésa D, Mitrea L, LoRe P, Vidal E, Levassor M
Service de Médecine interne A, CHU Dupuytren, Limoges.
Presse Med. 1999 Nov 6;28(34):1877-8.
Idiopathic capillary hyperpermeability syndrome is expressed by episodes of hypovolemic shock with normal consciousness.
A 61-year-old man had a history of recurrent shock. During the shock episodes, blood pressure was non-measurable and laboratory tests showed polycythemia, low serum protein and monoclonal immunoglobulinemia. The patient also developed pneumocystosis and had an infra-His block.
The final diagnosis was idiopathic capillary hyperpermeability syndrome. This rare syndrome associates recurrent shock with IgG monoclonal immunoglobulinemia and, at the time of the shock episodes, increased hematocrit with paradoxical fall in serum protein.