Abstract

Abstract A 6-year-old child gradually developed systemic lupus erythematosus (SLE). In the first titration 3 months after onset of the disease, a zero C′H50 titer was found, while SLE was not yet manifest. At that time, the lupus erythematosus (LE) cell test was still negative and there were no anti-nuclear factors (ANF) present. Immunoglobulins could not be demonstrated in the basal membrane of the skin. Fluorescence demonstrating immunoglobulin fixation in skin was for the first time positive 5 months after the beginning of the complaints, while the LE cell test and ANF fluorescence remained negative. ANF were first demonstrated at 10 months. The LE cell test remained negative. A C′H50 titer of 0 value was found throughout. Patient's sera were tested for C′1, C′2, C′4 and “C′3 complex” (EDTA serum); only absence of C′1 was consistently noted. The C′1 esterase inhibitor level of the serum was significantly increased varying from 150 to 300% above normal.

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