[Progressive septic granulomatosis (PSG)--a new clinical syndrome in pediatrics].
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Since the age of nine weeks a red haired girl suffered from purulent dermatitis and recurrent, systemic E. coli infections. She had an excessive hyperimmunoglobulinemia E, as well as impaired granulocyte adherence and chemotaxis. Though a sepsis was evident, the granulocytes exhibited a random FITC-Concanavalin A fluorescence. In spite of intensive treatment with various antibiotics and several granulocyte transfusions the child died at the age of 2 years and 11 months. As shown by the FITC-Concanavalin A distribution, the hyperimmunoglobulinemia E may have caused a decreased membrane fluidity causing the impaired adherence and chemotaxis. This could explain the pathophysiology of the Job's Syndrome.